JAX
Mouse Strain Datasheet - 003342
STOCK Hbatm1Paz Hbbtm1Tow Tg(HBA-HBBs)41Paz/J
Also Known As: Berkeley model, sickle cell anemia
Mice homozygous for the alpha-globin null allele, homozygous for the beta-globin null allele and carrying the sickle transgene (Hba0/0 Hbb0/0 Tg(Hu-miniLCRα1GγAγδβS) are called sickle cell mice (Berkeley model). These mice display the major genetic, hematologic and histopathologic features observed in humans with sickle cell anemia and may be useful in studying sickle cell disease.
Donating Investigator
Dr. Chris Paszty, Amgen, Inc.
Genetic overview
| Genetic Background | Generation |
|---|---|
|
N1F7G24
|
Hbatm1Paz
| Allele Type | Gene Symbol | Gene Name |
|---|---|---|
| Targeted (Null/Knockout) | Hba | hemoglobin alpha chain complex |
Hbbtm1Tow
| Allele Type | Gene Symbol | Gene Name |
|---|---|---|
| Targeted (Null/Knockout) | Hbb | hemoglobin beta chain complex |
Tg(HBA-HBBs)41Paz
| Allele Type |
|---|
| Transgenic (Humanized sequence, Inserted expressed sequence) |
Base Price
Starting at:
| $333.00 Domestic price for female |
| $666.00 Domestic price for breeder pair |
Detailed Description
These mice harbor
1) the Hbatm1Paz null mutation (also called Hba0 ; designed with both of the adult hemoglobin genes [α1 and α2] deleted),
2) the Hbbtm1Tow null mutation (also called Hbb0 ; designed with deletion of the entire hemoglobin beta-β1 [major] and the 5' portion of the hemoglobin beta-β2 [minor]),
3) the Tg(HBA-HBBs)41Paz transgene (also called Tg(Hu-miniLCRα1GγAγδβS); designed with the human sequences encoding the hemoglobin subunits alpha 1 (HBA1), gamma 2 (HBG2), gamma (HBG1), delta (HBD), and the beta sickle allele (HBBS), and the locus control region (LCR).
Mice homozygous for the alpha-globin null allele, homozygous for the beta-globin null allele and carrying the sickle transgene (Hba0/0 Hbb0/0 Tg(Hu-miniLCRα1GγAγδβS) are called sickle cell mice (Berkeley model). They exclusively express human sickle hemoglobin, and do not express mouse Hba or Hbb. Although chronically anemic, most of these mice survive for 2 to 9 months and are fertile. A significant percentage of sickle cell mice do not survive to adulthood. These mice display the major genetic, hematologic and histopathologic features observed in humans with sickle cell anemia; including irreversibly sickled red blood cells, anemia and multiorgan pathology. Typically, ~20% of sickling mutant mice die between weaning and 14 weeks of age.
Development
The Hbatm1Paz null mutation (also called Hba0) was designed by Dr. Chris Paszty to have both of the endogenous adult hemoglobin genes (α1 and α2) deleted and replaced with a neo cassette.
The Hbbtm1Tow null mutation (also called Hbb0) was designed by Dr. Timothy Townes to have the endogenous hemoglobin beta sequences (the entire hemoglobin beta-β1 [major] and the 5' portion of the hemoglobin beta-β2 [minor]) deleted and replaced with a neo cassette.
The Tg(HBA-HBBs)41Paz transgene (also called Tg(Hu-miniLCRα1GγAγδβS) was designed by Dr. Chris Paszty to have sequences encoding the human hemoglobin subunits alpha 1 (HBA1), gamma 2 (HBG2), gamma (HBG1), delta (HBD), and the beta sickle allele (HBBS), as well as the locus control region (LCR). The HBBS sequence contains an A-to-T transversion mutation in HBB codon six (amino acid change from glutamic acid to valine; E6V). The transgene promoter is multiple; from alpha and beta globin human loci. The transgene was injected into fertilized FVB/N mouse eggs.
Breeding the above mice together created the triple mutant mice - Tg(Hu-miniLCRα1GγAγδβS Hba0 Hbb0. The STOCK background of the triple mutant strain is a mixture of FVB/N, 129, DBA/2, Black Swiss and >50% C57BL/6 genomes. It was backcrossed to C57BL/6J one generation after importation to The Jackson Laboratory.
Expression Data
| Expressed Gene | HBA1, hemoglobin subunit alpha 1, human |
|---|---|
| Expressed Gene | HBB, hemoglobin subunit beta, human |
| Expressed Gene | HBD, hemoglobin subunit delta, human |
| Expressed Gene | HBG1, hemoglobin subunit gamma 1, human |
| Expressed Gene | HBG2, hemoglobin subunit gamma 2, human |
| Site of Expression |
Control Suggestions
- Homozygous for Hbatm1Paz, Heterozygous for Hbbtm1Tow, Hemizygous for Tg(HBA-HBBs)41Paz (non-sickling females from the colony)
Approximate Controls
Additional Information
Selected References
- Paszty C; Brion CM; Manci E; Witkowska HE; Stevens ME; Mohandas N; Rubin EM. 1997. Transgenic knockout mice with exclusively human sickle hemoglobin and sickle cell disease [see comments] Science 278(5339):876-8. PubMed: 9346488MGI: J:44161
Hbatm1Paz
Allele Symbol: Hbatm1Paz
| Allele Name | targeted mutation 1, Chris Paszty |
|---|---|
| Allele Type | Targeted (Null/Knockout) |
| Allele Synonym(s) | Hba0; Hba1-2del |
| Gene Symbol and Name | Hba, hemoglobin alpha chain complex |
| Gene Synonym(s) | |
| Strain of Origin | 129S7/SvEvBrd-Hprt<+> |
| Chromosome | 11 |
| Molecular Note | Both of the adult hemoglobin genes, alpha 1 and alpha 2, and the region between them were deleted and replaced with a neomycin resistance cassette by homologous recombination. |
| Mutations Made By | Dr. Chris Paszty, Amgen, Inc. |
Hbbtm1Tow
Allele Symbol: Hbbtm1Tow
| Allele Name | targeted mutation 1, Timothy Townes |
|---|---|
| Allele Type | Targeted (Null/Knockout) |
| Allele Synonym(s) | Hbb0; Hbbth3; Hbbtm1Tmt |
| Gene Symbol and Name | Hbb, hemoglobin beta chain complex |
| Gene Synonym(s) | |
| Strain of Origin | 129S2/SvPas |
| Chromosome | 7 |
| Molecular Note | A genomic fragment encompassing all of Hbb-b1 and a 5' portion of Hbb-b2 was replaced with a neo cassette inserted by homologous recombination. |
| Mutations Made By | Timothy Townes, University of Alabama School of Medicine |
Tg(HBA-HBBs)41Paz
Allele Symbol: Tg(HBA-HBBs)41Paz
| Allele Name | transgene insertion 41, Chris Paszty |
|---|---|
| Allele Type | Transgenic (Humanized sequence, Inserted expressed sequence) |
| Allele Synonym(s) | Tg(Hu-miniLCRalpha1GgammaAgammadeltabetaS); miniLCRalpha1GgammaAgammadeltabetaS |
| Gene Symbol and Name | Tg(HBA-HBBs)41Paz, transgene insertion 41, Chris Paszty |
| Gene Synonym(s) | BERK; Tg(Hu-miniLCRalpha1GgammaAgammadeltabetaS) |
| Promoter | HBA1, hemoglobin subunit alpha 1, human |
| Promoter | HBB, hemoglobin subunit beta, human |
| Expressed Gene | HBA1, hemoglobin subunit alpha 1, human |
| Expressed Gene | HBB, hemoglobin subunit beta, human |
| Expressed Gene | HBD, hemoglobin subunit delta, human |
| Expressed Gene | HBG1, hemoglobin subunit gamma 1, human |
| Expressed Gene | HBG2, hemoglobin subunit gamma 2, human |
| Strain of Origin | FVB/N |
| Chromosome | UN |
| General Note | In conjunction with Hbatm1Paz and Hbbtm1Tmt, transgenic mice express exclusively human sickle hemoglobin. These mice do not express mouse Hba and Hbb, but do express human HBA and HBB. Although chronically anemic, most of these mice survive for 2 to 9 months and are fertile. A significant percentage of sickle cell mice do not survive to adulthood. These mice display the major genetic, hematologic and histopathologic features found in humans with sickle cell disease: irreversibly sickled red cells, anemia, and multiorgan pathology. |
| Molecular Note | The transgene contains sequences encoding the human proteins HBA1 (hemoglobin, alpha 1), HBG2 (hemoglobin, gamma G, fetal component), HBG1 (hemoglobin, gamma A, fetal component), HBD (hemoglobin, delta), and HBB S (hemoglobin, beta, sickle allele), and the locus control region (LCR). The HBBS allele contains an A to T transversion mutation in the sixth codon of HBB which causes an amino acid change from Glu to Val. The transgene promoter is multiple: from alpha and beta globin human loci. Transgenic mice express human alpha hemoglobin, gamma hemoglobin, and sickle cell hemoglobin. |
| Mutations Made By | Dr. Chris Paszty, Amgen, Inc. |
Disease Terms
Research Areas By Genotype
This mouse can be used to support research in many areas including:
- Hematological Research
- Sickle Cell Anemia
Genotype: HBB related
- Hematological Research
- Hemoglobin Defects
Genotype: Hbatm1Paz related
- Hematological Research
- Hemoglobin Defects
Genotype: Hbbtm1Tow related
- Hematological Research
- Hemoglobin Defects
Mammalian Phenotype Terms by Genotype
Genotype: Hbatm1Paz/Hbatm1Paz Hbbtm1Tow/Hbbtm1Tow Tg(HBA-HBBs)41Paz/0
involves: 129 * Black Swiss * C57BL/6 * DBA/2 * FVB/N
reproductive system phenotype
- abnormal penile erection
Genotype: Hbatm1Paz/Hbatm1Paz Hbbtm1Tow/Hbbtm1Tow Tg(HBA-HBBs)41Paz/?
involves: 129S2/SvPas * 129S7/SvEvBrd * Black Swiss * C57BL/6 * DBA/2* FVB/N
mortality/aging
- neonatal lethality, incomplete penetrance
- many pups turn purple and die within a few hours of birth, however, mice that survive live to become adults (reaching at least 7 months of age) (MGI Ref ID J:44161)
hematopoietic system phenotype
- abnormal erythrocyte morphology
- erythrocytes have increased dynamic rigidity (MGI Ref ID J:44161)
- abnormal erythrocyte osmotic lysis
- erythrocytes have decreased osmotic fragility (MGI Ref ID J:44161)
- abnormal hemoglobin
- erythrocytes contain an excess of alpha globin chain synthesis (MGI Ref ID J:44161)
- anemia
- (MGI Ref ID J:44161)
- anisopoikilocytosis
- sickle cell shapes are observed at a frequency of 5-10% in oxygenated blood (MGI Ref ID J:44161)
- decreased hematocrit
- average hematocrit levels are 65% of control at 3-7 months of age (MGI Ref ID J:44161)
- decreased mean corpuscular hemoglobin
- (MGI Ref ID J:44161)
- decreased mean corpuscular hemoglobin concentration
- (MGI Ref ID J:44161)
- decreased mean corpuscular volume
- (MGI Ref ID J:44161)
- increased spleen weight
- weight is increased 13-fold as compared to control (MGI Ref ID J:44161)
- reticulocytosis
- reticulocyte counts are elevated at 3-7 months of age (MGI Ref ID J:44161)
- spleen vascular congestion
- congested sinusoidal channels are observed in spleen (MGI Ref ID J:44161)
homeostasis/metabolism phenotype
- hypoxia
- cause of death in some newborn mice (MGI Ref ID J:44161)
- increased kidney iron level
- iron deposits found in the tubular epithelium of the kidney (MGI Ref ID J:44161)
- increased liver iron level
- iron deposits found in Kupffer cells of liver (MGI Ref ID J:44161)
cardiovascular system phenotype
- abnormal kidney blood vessel morphology
- kidney infarction (MGI Ref ID J:44161)
- abnormal liver vasculature morphology
- liver infarction (MGI Ref ID J:44161)
- abnormal lung vasculature morphology
- lung infarction (MGI Ref ID J:44161)
- increased heart weight
- weight is increased 2-fold as compared to control (MGI Ref ID J:44161)
- spleen vascular congestion
- congested sinusoidal channels are observed in spleen (MGI Ref ID J:44161)
renal/urinary system phenotype
- abnormal kidney blood vessel morphology
- kidney infarction (MGI Ref ID J:44161)
- increased kidney iron level
- iron deposits found in the tubular epithelium of the kidney (MGI Ref ID J:44161)
- increased kidney weight
- weight is increased 2-fold as compared to control (MGI Ref ID J:44161)
- kidney atrophy
- (MGI Ref ID J:44161)
- kidney cysts
- (MGI Ref ID J:44161)
- renal fibrosis
- (MGI Ref ID J:44161)
immune system phenotype
- increased spleen weight
- weight is increased 13-fold as compared to control (MGI Ref ID J:44161)
- spleen vascular congestion
- congested sinusoidal channels are observed in spleen (MGI Ref ID J:44161)
liver/biliary system phenotype
- abnormal liver vasculature morphology
- liver infarction (MGI Ref ID J:44161)
- increased liver iron level
- iron deposits found in Kupffer cells of liver (MGI Ref ID J:44161)
respiratory system phenotype
- abnormal lung vasculature morphology
- lung infarction (MGI Ref ID J:44161)
References
- Paszty C; Brion CM; Manci E; Witkowska HE; Stevens ME; Mohandas N; Rubin EM. 1997. Transgenic knockout mice with exclusively human sickle hemoglobin and sickle cell disease [see comments] Science 278(5339):876-8. PubMed: 9346488MGI: J:44161
Additional References
- Barinaga M. 1997. Mutant mice mimic human sickle cell anemia [news; comment] Science 278(5339):803-4. PubMed: 9381190MGI: J:44159
- Ciavatta DJ; Ryan TM; Farmer SC; Townes TM. 1995. Mouse model of human beta zero thalassemia: targeted deletion of the mouse beta maj- and beta min-globin genes in embryonic stem cells. Proc Natl Acad Sci U S A 92(20):9259-63. PubMed: 7568113MGI: J:29087
- Ryan TM; Ciavatta DJ; Townes TM. 1997. Knockout-transgenic mouse model of sickle cell disease [see comments] Science 278(5339):873-6. PubMed: 9346487MGI: J:44160
Additional - Hbatm1Paz related
- Al-Hasani K; Vadolas J; Knaupp AS; Wardan H; Voullaire L; Williamson R; Ioannou PA. 2005. A 191-kb genomic fragment containing the human alpha-globin locus can rescue alpha-thalassemic mice. Mamm Genome 16(11):847-53. PubMed: 16284800MGI: J:103573
- Archer DR; Stiles JK; Newman GW; Quarshie A; Hsu LL; Sayavongsa P; Perry J; Jackson EM; Hibbert JM. 2008. C-reactive protein and interleukin-6 are decreased in transgenic sickle cell mice fed a high protein diet. J Nutr 138(6):1148-52. PubMed: 18492848MGI: J:136372
- Arumugam PI; Mullins ES; Shanmukhappa SK; Monia BP; Loberg A; Shaw MA; Rizvi T; Wansapura J; Degen JL; Malik P. 2015. Genetic diminution of circulating prothrombin ameliorates multiorgan pathologies in sickle cell disease mice. Blood 126(15):1844-55. PubMed: 26286849MGI: J:230254
- Bakeer N; James J; Roy S; Wansapura J; Shanmukhappa SK; Lorenz JN; Osinska H; Backer K; Huby AC; Shrestha A; Niss O; Fleck R; Quinn CT; Taylor MD; Purevjav E; Aronow BJ; Towbin JA; Malik P. 2016. Sickle cell anemia mice develop a unique cardiomyopathy with restrictive physiology. Proc Natl Acad Sci U S A 113(35):E5182-91. PubMed: 27503873MGI: J:235608
- Banerjee T; Kuypers FA. 2004. Reactive oxygen species and phosphatidylserine externalization in murine sickle red cells. Br J Haematol 124(3):391-402. PubMed: 14717789MGI: J:88553
- Barazia A; Li J; Kim K; Shabrani N; Cho J. 2015. Hydroxyurea with AKT2 inhibition decreases vaso-occlusive events in sickle cell disease mice. Blood 126(22):2511-7. PubMed: 26265698MGI: J:230085
- Barinaga M. 1997. Mutant mice mimic human sickle cell anemia [news; comment] Science 278(5339):803-4. PubMed: 9381190MGI: J:44159
- Belcher JD; Mahaseth H; Welch TE; Otterbein LE; Hebbel RP; Vercellotti GM. 2006. Heme oxygenase-1 is a modulator of inflammation and vaso-occlusion in transgenic sickle mice. J Clin Invest 116(3):808-16. PubMed: 16485041MGI: J:106486
- Bivalacqua TJ; Musicki B; Hsu LL; Berkowitz DE; Champion HC; Burnett AL. 2013. Sildenafil citrate-restored eNOS and PDE5 regulation in sickle cell mouse penis prevents priapism via control of oxidative/nitrosative stress. PLoS One 8(7):e68028. PubMed: 23844149MGI: J:204301
- Champion HC; Bivalacqua TJ; Takimoto E; Kass DA; Burnett AL. 2005. Phosphodiesterase-5A dysregulation in penile erectile tissue is a mechanism of priapism. Proc Natl Acad Sci U S A 102(5):1661-6. PubMed: 15668387MGI: J:96103
- Chang J; Patton JT; Sarkar A; Ernst B; Magnani JL; Frenette PS. 2010. GMI-1070, a novel pan-selectin antagonist, reverses acute vascular occlusions in sickle cell mice. Blood 116(10):1779-86. PubMed: 20508165MGI: J:164535
- Chang JC; Ye L; Kan YW. 2006. Correction of the sickle cell mutation in embryonic stem cells. Proc Natl Acad Sci U S A 103(4):1036-40. PubMed: 16407095MGI: J:105707
- Chantrathammachart P; Mackman N; Sparkenbaugh E; Wang JG; Parise LV; Kirchhofer D; Key NS; Pawlinski R. 2012. Tissue factor promotes activation of coagulation and inflammation in a mouse model of sickle cell disease. Blood 120(3):636-46. PubMed: 22661702MGI: J:189104
- Chen G; Zhang D; Fuchs TA; Manwani D; Wagner DD; Frenette PS. 2014. Heme-induced neutrophil extracellular traps contribute to the pathogenesis of sickle cell disease. Blood 123(24):3818-27. PubMed: 24620350MGI: J:210886
- Dasgupta T; Fabry ME; Kaul DK. 2010. Antisickling property of fetal hemoglobin enhances nitric oxide bioavailability and ameliorates organ oxidative stress in transgenic-knockout sickle mice. Am J Physiol Regul Integr Comp Physiol 298(2):R394-402. PubMed: 20007516MGI: J:156179
- Dasgupta T; Hebbel RP; Kaul DK. 2006. Protective effect of arginine on oxidative stress in transgenic sickle mouse models. Free Radic Biol Med 41(12):1771-80. PubMed: 17157180MGI: J:116710
- Eiymo Mwa Mpollo MS; Brandt EB; Shanmukhappa SK; Arumugam PI; Tiwari S; Loberg A; Pillis D; Rizvi T; Lindsey M; Jonck B; Carmeliet P; Kalra VK; Le Cras TD; Ratner N; Wills-Karp M; Hershey GK; Malik P. 2016. Placenta growth factor augments airway hyperresponsiveness via leukotrienes and IL-13. J Clin Invest 126(2):571-84. PubMed: 26690703MGI: J:231182
- Fabry ME; Suzuka SM; Weinberg RS; Lawrence C; Factor SM; Gilman JG; Costantini F; Nagel RL. 2001. Second generation knockout sickle mice: the effect of HbF. Blood 97(2):410-8. PubMed: 11154217MGI: J:66961
- Gavins FN; Russell J; Senchenkova EL; De Almeida Paula L; Damazo AS; Esmon CT; Kirchhofer D; Hebbel RP; Granger DN. 2011. Mechanisms of enhanced thrombus formation in cerebral microvessels of mice expressing hemoglobin-S. Blood 117(15):4125-33. PubMed: 21304105MGI: J:172840
- Gutsaeva DR; Montero-Huerta P; Parkerson JB; Yerigenahally SD; Ikuta T; Head CA. 2014. Molecular mechanisms underlying synergistic adhesion of sickle red blood cells by hypoxia and low nitric oxide bioavailability. Blood 123(12):1917-26. PubMed: 24429338MGI: J:209498
- Gutsaeva DR; Parkerson JB; Yerigenahally SD; Kurz JC; Schaub RG; Ikuta T; Head CA. 2011. Inhibition of cell adhesion by anti-P-selectin aptamer: a new potential therapeutic agent for sickle cell disease. Blood 117(2):727-35. PubMed: 20926770MGI: J:168427
- Hanson MS; Xu H; Flewelen TC; Holzhauer SL; Retherford D; Jones DW; Frei AC; Pritchard KA Jr; Hillery CA; Hogg N; Wandersee NJ. 2013. A novel hemoglobin-binding peptide reduces cell-free hemoglobin in murine hemolytic anemia. Am J Physiol Heart Circ Physiol 304(2):H328-36. PubMed: 23125208MGI: J:192837
- Hebbel RP; Vercellotti GM; Pace BS; Solovey AN; Kollander R; Abanonu CF; Nguyen J; Vineyard JV; Belcher JD; Abdulla F; Osifuye S; Eaton JW; Kelm RJ Jr; Slungaard A. 2010. The HDAC inhibitors trichostatin A and suberoylanilide hydroxamic acid exhibit multiple modalities of benefit for the vascular pathobiology of sickle transgenic mice. Blood 115(12):2483-90. PubMed: 20053759MGI: J:159262
- Hidalgo A; Chang J; Jang JE; Peired AJ; Chiang EY; Frenette PS. 2009. Heterotypic interactions enabled by polarized neutrophil microdomains mediate thromboinflammatory injury. Nat Med 15(4):384-91. PubMed: 19305412MGI: J:149370
- Hsu LL; Champion HC; Campbell-Lee SA; Bivalacqua TJ; Manci EA; Diwan BA; Schimel DM; Cochard AE; Wang X; Schechter AN; Noguchi CT; Gladwin MT. 2007. Hemolysis in sickle cell mice causes pulmonary hypertension due to global impairment in nitric oxide bioavailability. Blood 109(7):3088-98. PubMed: 17158223MGI: J:145347
- Ieremia J; Blau CA. 2002. Limitations of a mouse model of sickle cell anemia. Blood Cells Mol Dis 28(2):146-51. PubMed: 12064910MGI: J:128113
- Kaul DK; Fabry ME; Suzuka SM; Zhang X. 2013. Antisickling fetal hemoglobin reduces hypoxia-inducible factor-1alpha expression in normoxic sickle mice: microvascular implications. Am J Physiol Heart Circ Physiol 304(1):H42-50. PubMed: 23125209MGI: J:192836
- Kaul DK; Liu XD; Chang HY; Nagel RL; Fabry ME. 2004. Effect of fetal hemoglobin on microvascular regulation in sickle transgenic-knockout mice. J Clin Invest 114(8):1136-45. PubMed: 15489961MGI: J:93424
- Kiefmann R; Rifkind JM; Nagababu E; Bhattacharya J. 2008. Red blood cells induce hypoxic lung inflammation. Blood 111(10):5205-14. PubMed: 18270324MGI: J:135569
- Kohli DR; Li Y; Khasabov SG; Gupta P; Kehl LJ; Ericson ME; Nguyen J; Gupta V; Hebbel RP; Simone DA; Gupta K. 2010. Pain-related behaviors and neurochemical alterations in mice expressing sickle hemoglobin: modulation by cannabinoids. Blood 116(3):456-65. PubMed: 20304807MGI: J:162824
- Lagoda G; Sezen SF; Hurt KJ; Cabrini MR; Mohanty DK; Burnett AL. 2014. Sustained nitric oxide (NO)-releasing compound reverses dysregulated NO signal transduction in priapism. FASEB J 28(1):76-84. PubMed: 24076963MGI: J:206623
- Lebensburger JD; Howard T; Hu Y; Pestina TI; Gao G; Johnson M; Zakharenko SS; Ware RE; Tuomanen EI; Persons DA; Rosch JW. 2012. Hydroxyurea therapy of a murine model of sickle cell anemia inhibits the progression of pneumococcal disease by down-modulating E-selectin. Blood 119(8):1915-21. PubMed: 22130804MGI: J:181749
- Levasseur DN; Ryan TM; Pawlik KM; Townes TM. 2003. Correction of a mouse model of sickle cell disease: lentiviral/antisickling beta-globin gene transduction of unmobilized, purified hematopoietic stem cells. Blood 102(13):4312-9. PubMed: 12933581MGI: J:134982
- Li J; Kim K; Hahm E; Molokie R; Hay N; Gordeuk VR; Du X; Cho J. 2014. Neutrophil AKT2 regulates heterotypic cell-cell interactions during vascular inflammation. J Clin Invest 124(4):1483-96. PubMed: 24642468MGI: J:209590
- Liu S; McConnell SC; Ryan TM. 2013. Erythropoiesis in the absence of adult hemoglobin. Mol Cell Biol 33(11):2241-51. PubMed: 23530053MGI: J:204029
- Manci EA; Hillery CA; Bodian CA; Zhang ZG; Lutty GA; Coller BS. 2006. Pathology of Berkeley sickle cell mice: similarities and differences with human sickle cell disease. Blood 107(4):1651-8. PubMed: 16166585MGI: J:129356
- Meiler SE; Wade M; Kutlar F; Yerigenahally SD; Xue Y; Moutouh-de Parseval LA; Corral LG; Swerdlow PS; Kutlar A. 2011. Pomalidomide augments fetal hemoglobin production without the myelosuppressive effects of hydroxyurea in transgenic sickle cell mice. Blood 118(4):1109-12. PubMed: 21536862MGI: J:174901
- Mi T; Abbasi S; Zhang H; Uray K; Chunn JL; Xia LW; Molina JG; Weisbrodt NW; Kellems RE; Blackburn MR; Xia Y. 2008. Excess adenosine in murine penile erectile tissues contributes to priapism via A2B adenosine receptor signaling. J Clin Invest 118(4):1491-501. PubMed: 18340377MGI: J:135978
- Musicki B; Zhang Y; Chen H; Brown TR; Zirkin BR; Burnett AL. 2015. Mechanism of testosterone deficiency in the transgenic sickle cell mouse. PLoS One 10(5):e0128694. PubMed: 26023917MGI: J:237731
- Ning C; Wen J; Zhang Y; Dai Y; Wang W; Zhang W; Qi L; Grenz A; Eltzschig HK; Blackburn MR; Kellems RE; Xia Y. 2014. Excess adenosine A2B receptor signaling contributes to priapism through HIF-1alpha mediated reduction of PDE5 gene expression. FASEB J 28(6):2725-35. PubMed: 24614760MGI: J:216540
- Noguchi CT; Gladwin M; Diwan B; Merciris P; Smith R; Yu X; Buzard G; Fitzhugh A; Keefer LK; Schechter AN; Mohandas N. 2001. Pathophysiology of a sickle cell trait mouse model: human alpha(beta)(S) transgenes with one mouse beta-globin allele. Blood Cells Mol Dis 27(6):971-7. PubMed: 11831863MGI: J:128124
- Paszty C; Mohandas N; Stevens ME; Loring JF; Liebhaber SA; Brion CM; Rubin EM. 1995. Lethal alpha-thalassaemia created by gene targeting in mice and its genetic rescue. Nat Genet 11(1):33-9. PubMed: 7550311MGI: J:28392
- Peranteau WH; Hayashi S; Abdulmalik O; Chen Q; Merchant A; Asakura T; Flake AW. 2015. Correction of murine hemoglobinopathies by prenatal tolerance induction and postnatal nonmyeloablative allogeneic BM transplants. Blood 126(10):1245-54. PubMed: 26124498MGI: J:226447
- Prado GN; Romero JR; Rivera A. 2013. Endothelin-1 receptor antagonists regulate cell surface-associated protein disulfide isomerase in sickle cell disease. FASEB J 27(11):4619-29. PubMed: 23913858MGI: J:203687
- Pritchard KA Jr; Feroah TR; Nandedkar SD; Holzhauer SL; Hutchins W; Schulte ML; Strunk RC; Debaun MR; Hillery CA. 2012. Effects of experimental asthma on inflammation and lung mechanics in sickle cell mice. Am J Respir Cell Mol Biol 46(3):389-96. PubMed: 22033263MGI: J:194579
- Pritchard KA Jr; Ou J; Ou Z; Shi Y; Franciosi JP; Signorino P; Kaul S; Ackland-Berglund C; Witte K; Holzhauer S; Mohandas N; Guice KS; Oldham KT; Hillery CA. 2004. Hypoxia-induced acute lung injury in murine models of sickle cell disease. Am J Physiol Lung Cell Mol Physiol 286(4):L705-14. PubMed: 12972407MGI: J:134696
- Romero JR; Suzuka SM; Nagel RL; Fabry ME. 2004. Expression of HbC and HbS, but not HbA, results in activation of K-Cl cotransport activity in transgenic mouse red cells. Blood 103(6):2384-90. PubMed: 14615383MGI: J:88563
- Russell JE; Liebhaber SA. 1998. Reversal of lethal alpha- and beta-thalassemias in mice by expression of human embryonic globins. Blood 92(9):3057-63. PubMed: 9787139MGI: J:114200
- Ryan TM; Ciavatta DJ; Townes TM. 1997. Knockout-transgenic mouse model of sickle cell disease [see comments] Science 278(5339):873-6. PubMed: 9346487MGI: J:44160
- Solovey A; Kollander R; Shet A; Milbauer LC; Choong S; Panoskaltsis-Mortari A; Blazar BR; Kelm RJ Jr; Hebbel RP. 2004. Endothelial cell expression of tissue factor in sickle mice is augmented by hypoxia/reoxygenation and inhibited by lovastatin. Blood 104(3):840-6. PubMed: 15073034MGI: J:92287
- Sparkenbaugh EM; Chantrathammachart P; Chandarajoti K; Mackman N; Key NS; Pawlinski R. 2016. Thrombin-independent contribution of tissue factor to inflammation and cardiac hypertrophy in a mouse model of sickle cell disease. Blood 127(10):1371-3. PubMed: 26817955MGI: J:232634
- Sparkenbaugh EM; Chantrathammachart P; Mickelson J; van Ryn J; Hebbel RP; Monroe DM; Mackman N; Key NS; Pawlinski R. 2014. Differential contribution of FXa and thrombin to vascular inflammation in a mouse model of sickle cell disease. Blood 123(11):1747-56. PubMed: 24449213MGI: J:209655
- Sun K; Zhang Y; Bogdanov MV; Wu H; Song A; Li J; Dowhan W; Idowu M; Juneja HS; Molina JG; Blackburn MR; Kellems RE; Xia Y. 2015. Elevated adenosine signaling via adenosine A2B receptor induces normal and sickle erythrocyte sphingosine kinase 1 activity. Blood 125(10):1643-52. PubMed: 25587035MGI: J:221918
- Sundaram N; Tailor A; Mendelsohn L; Wansapura J; Wang X; Higashimoto T; Pauciulo MW; Gottliebson W; Kalra VK; Nichols WC; Kato GJ; Malik P. 2010. High levels of placenta growth factor in sickle cell disease promote pulmonary hypertension. Blood 116(1):109-12. PubMed: 20335221MGI: J:162823
- Vincent L; Vang D; Nguyen J; Gupta M; Luk K; Ericson ME; Simone DA; Gupta K. 2013. Mast cell activation contributes to sickle cell pathobiology and pain in mice. Blood 122(11):1853-62. PubMed: 23775718MGI: J:202290
- Voon HP; Wardan H; Vadolas J. 2007. Co-inheritance of alpha- and beta-thalassaemia in mice ameliorates thalassaemic phenotype. Blood Cells Mol Dis 39(2):184-8. PubMed: 17493845MGI: J:141719
- Wallace HA; Marques-Kranc F; Richardson M; Luna-Crespo F; Sharpe JA; Hughes J; Wood WG; Higgs DR; Smith AJ. 2007. Manipulating the mouse genome to engineer precise functional syntenic replacements with human sequence. Cell 128(1):197-209. PubMed: 17218265MGI: J:117887
- Wang L; Almeida LE; de Souza Batista CM; Khaibullina A; Xu N; Albani S; Guth KA; Seo JS; Quezado M; Quezado ZM. 2016. Cognitive and behavior deficits in sickle cell mice are associated with profound neuropathologic changes in hippocampus and cerebellum. Neurobiol Dis 85:60-72. PubMed: 26462816MGI: J:228649
- Wen J; Jiang X; Dai Y; Zhang Y; Tang Y; Sun H; Mi T; Phatarpekar PV; Kellems RE; Blackburn MR; Xia Y. 2010. Increased adenosine contributes to penile fibrosis, a dangerous feature of priapism, via A2B adenosine receptor signaling. FASEB J 24(3):740-9. PubMed: 19858092MGI: J:158034
- Whitney JB; Russell ES. 1978. New mutants and biochemical variants: Alpha thalassemia Mouse News Lett 58:47-48. MGI: J:45721
- Wood KC; Hebbel RP; Granger DN. 2004. Endothelial cell P-selectin mediates a proinflammatory and prothrombogenic phenotype in cerebral venules of sickle cell transgenic mice. Am J Physiol Heart Circ Physiol 286(5):H1608-14. PubMed: 14704223MGI: J:95603
- Wood KC; Hebbel RP; Granger DN. 2005. Endothelial cell NADPH oxidase mediates the cerebral microvascular dysfunction in sickle cell transgenic mice. FASEB J 19(8):989-91. PubMed: 15923406MGI: J:128246
- Xu J; Peng C; Sankaran VG; Shao Z; Esrick EB; Chong BG; Ippolito GC; Fujiwara Y; Ebert BL; Tucker PW; Orkin SH. 2011. Correction of sickle cell disease in adult mice by interference with fetal hemoglobin silencing. Science 334(6058):993-6. PubMed: 21998251MGI: J:177861
- Zhang Y; Dai Y; Wen J; Zhang W; Grenz A; Sun H; Tao L; Lu G; Alexander DC; Milburn MV; Carter-Dawson L; Lewis DE; Zhang W; Eltzschig HK; Kellems RE; Blackburn MR; Juneja HS; Xia Y. 2011. Detrimental effects of adenosine signaling in sickle cell disease. Nat Med 17(1):79-86. PubMed: 21170046MGI: J:168473
- Zhao Y; Schwartz EA; Palmer GM; Zennadi R. 2016. MEK1/2 inhibitors reverse acute vascular occlusion in mouse models of sickle cell disease. FASEB J 30(3):1171-86. PubMed: 26631480MGI: J:230900
- de Jong K; Emerson RK; Butler J; Bastacky J; Mohandas N; Kuypers FA. 2001. Short survival of phosphatidylserine-exposing red blood cells in murine sickle cell anemia. Blood 98(5):1577-84. PubMed: 11520810MGI: J:131515
Additional - Hbbtm1Tow related
- Abdulmalik O; Safo MK; Chen Q; Yang J; Brugnara C; Ohene-Frempong K; Abraham DJ; Asakura T. 2005. 5-hydroxymethyl-2-furfural modifies intracellular sickle haemoglobin and inhibits sickling of red blood cells. Br J Haematol 128(4):552-61. PubMed: 15686467MGI: J:96026
- Archer DR; Stiles JK; Newman GW; Quarshie A; Hsu LL; Sayavongsa P; Perry J; Jackson EM; Hibbert JM. 2008. C-reactive protein and interleukin-6 are decreased in transgenic sickle cell mice fed a high protein diet. J Nutr 138(6):1148-52. PubMed: 18492848MGI: J:136372
- Arumugam PI; Mullins ES; Shanmukhappa SK; Monia BP; Loberg A; Shaw MA; Rizvi T; Wansapura J; Degen JL; Malik P. 2015. Genetic diminution of circulating prothrombin ameliorates multiorgan pathologies in sickle cell disease mice. Blood 126(15):1844-55. PubMed: 26286849MGI: J:230254
- Bakeer N; James J; Roy S; Wansapura J; Shanmukhappa SK; Lorenz JN; Osinska H; Backer K; Huby AC; Shrestha A; Niss O; Fleck R; Quinn CT; Taylor MD; Purevjav E; Aronow BJ; Towbin JA; Malik P. 2016. Sickle cell anemia mice develop a unique cardiomyopathy with restrictive physiology. Proc Natl Acad Sci U S A 113(35):E5182-91. PubMed: 27503873MGI: J:235608
- Banerjee T; Kuypers FA. 2004. Reactive oxygen species and phosphatidylserine externalization in murine sickle red cells. Br J Haematol 124(3):391-402. PubMed: 14717789MGI: J:88553
- Barazia A; Li J; Kim K; Shabrani N; Cho J. 2015. Hydroxyurea with AKT2 inhibition decreases vaso-occlusive events in sickle cell disease mice. Blood 126(22):2511-7. PubMed: 26265698MGI: J:230085
- Barinaga M. 1997. Mutant mice mimic human sickle cell anemia [news; comment] Science 278(5339):803-4. PubMed: 9381190MGI: J:44159
- Belcher JD; Mahaseth H; Welch TE; Otterbein LE; Hebbel RP; Vercellotti GM. 2006. Heme oxygenase-1 is a modulator of inflammation and vaso-occlusion in transgenic sickle mice. J Clin Invest 116(3):808-16. PubMed: 16485041MGI: J:106486
- Bivalacqua TJ; Musicki B; Hsu LL; Berkowitz DE; Champion HC; Burnett AL. 2013. Sildenafil citrate-restored eNOS and PDE5 regulation in sickle cell mouse penis prevents priapism via control of oxidative/nitrosative stress. PLoS One 8(7):e68028. PubMed: 23844149MGI: J:204301
- Champion HC; Bivalacqua TJ; Takimoto E; Kass DA; Burnett AL. 2005. Phosphodiesterase-5A dysregulation in penile erectile tissue is a mechanism of priapism. Proc Natl Acad Sci U S A 102(5):1661-6. PubMed: 15668387MGI: J:96103
- Chang J; Patton JT; Sarkar A; Ernst B; Magnani JL; Frenette PS. 2010. GMI-1070, a novel pan-selectin antagonist, reverses acute vascular occlusions in sickle cell mice. Blood 116(10):1779-86. PubMed: 20508165MGI: J:164535
- Chang JC; Ye L; Kan YW. 2006. Correction of the sickle cell mutation in embryonic stem cells. Proc Natl Acad Sci U S A 103(4):1036-40. PubMed: 16407095MGI: J:105707
- Chantrathammachart P; Mackman N; Sparkenbaugh E; Wang JG; Parise LV; Kirchhofer D; Key NS; Pawlinski R. 2012. Tissue factor promotes activation of coagulation and inflammation in a mouse model of sickle cell disease. Blood 120(3):636-46. PubMed: 22661702MGI: J:189104
- Chen G; Zhang D; Fuchs TA; Manwani D; Wagner DD; Frenette PS. 2014. Heme-induced neutrophil extracellular traps contribute to the pathogenesis of sickle cell disease. Blood 123(24):3818-27. PubMed: 24620350MGI: J:210886
- Ciavatta DJ; Ryan TM; Farmer SC; Townes TM. 1995. Mouse model of human beta zero thalassemia: targeted deletion of the mouse beta maj- and beta min-globin genes in embryonic stem cells. Proc Natl Acad Sci U S A 92(20):9259-63. PubMed: 7568113MGI: J:29087
- Dasgupta T; Fabry ME; Kaul DK. 2010. Antisickling property of fetal hemoglobin enhances nitric oxide bioavailability and ameliorates organ oxidative stress in transgenic-knockout sickle mice. Am J Physiol Regul Integr Comp Physiol 298(2):R394-402. PubMed: 20007516MGI: J:156179
- Dasgupta T; Hebbel RP; Kaul DK. 2006. Protective effect of arginine on oxidative stress in transgenic sickle mouse models. Free Radic Biol Med 41(12):1771-80. PubMed: 17157180MGI: J:116710
- Eiymo Mwa Mpollo MS; Brandt EB; Shanmukhappa SK; Arumugam PI; Tiwari S; Loberg A; Pillis D; Rizvi T; Lindsey M; Jonck B; Carmeliet P; Kalra VK; Le Cras TD; Ratner N; Wills-Karp M; Hershey GK; Malik P. 2016. Placenta growth factor augments airway hyperresponsiveness via leukotrienes and IL-13. J Clin Invest 126(2):571-84. PubMed: 26690703MGI: J:231182
- Gavins FN; Russell J; Senchenkova EL; De Almeida Paula L; Damazo AS; Esmon CT; Kirchhofer D; Hebbel RP; Granger DN. 2011. Mechanisms of enhanced thrombus formation in cerebral microvessels of mice expressing hemoglobin-S. Blood 117(15):4125-33. PubMed: 21304105MGI: J:172840
- Gutsaeva DR; Parkerson JB; Yerigenahally SD; Kurz JC; Schaub RG; Ikuta T; Head CA. 2011. Inhibition of cell adhesion by anti-P-selectin aptamer: a new potential therapeutic agent for sickle cell disease. Blood 117(2):727-35. PubMed: 20926770MGI: J:168427
- Hanson MS; Xu H; Flewelen TC; Holzhauer SL; Retherford D; Jones DW; Frei AC; Pritchard KA Jr; Hillery CA; Hogg N; Wandersee NJ. 2013. A novel hemoglobin-binding peptide reduces cell-free hemoglobin in murine hemolytic anemia. Am J Physiol Heart Circ Physiol 304(2):H328-36. PubMed: 23125208MGI: J:192837
- Hebbel RP; Vercellotti GM; Pace BS; Solovey AN; Kollander R; Abanonu CF; Nguyen J; Vineyard JV; Belcher JD; Abdulla F; Osifuye S; Eaton JW; Kelm RJ Jr; Slungaard A. 2010. The HDAC inhibitors trichostatin A and suberoylanilide hydroxamic acid exhibit multiple modalities of benefit for the vascular pathobiology of sickle transgenic mice. Blood 115(12):2483-90. PubMed: 20053759MGI: J:159262
- Hidalgo A; Chang J; Jang JE; Peired AJ; Chiang EY; Frenette PS. 2009. Heterotypic interactions enabled by polarized neutrophil microdomains mediate thromboinflammatory injury. Nat Med 15(4):384-91. PubMed: 19305412MGI: J:149370
- Hsu LL; Champion HC; Campbell-Lee SA; Bivalacqua TJ; Manci EA; Diwan BA; Schimel DM; Cochard AE; Wang X; Schechter AN; Noguchi CT; Gladwin MT. 2007. Hemolysis in sickle cell mice causes pulmonary hypertension due to global impairment in nitric oxide bioavailability. Blood 109(7):3088-98. PubMed: 17158223MGI: J:145347
- Ieremia J; Blau CA. 2002. Limitations of a mouse model of sickle cell anemia. Blood Cells Mol Dis 28(2):146-51. PubMed: 12064910MGI: J:128113
- Kaul DK; Fabry ME; Suzuka SM; Zhang X. 2013. Antisickling fetal hemoglobin reduces hypoxia-inducible factor-1alpha expression in normoxic sickle mice: microvascular implications. Am J Physiol Heart Circ Physiol 304(1):H42-50. PubMed: 23125209MGI: J:192836
- Kaul DK; Liu XD; Chang HY; Nagel RL; Fabry ME. 2004. Effect of fetal hemoglobin on microvascular regulation in sickle transgenic-knockout mice. J Clin Invest 114(8):1136-45. PubMed: 15489961MGI: J:93424
- Kiefmann R; Rifkind JM; Nagababu E; Bhattacharya J. 2008. Red blood cells induce hypoxic lung inflammation. Blood 111(10):5205-14. PubMed: 18270324MGI: J:135569
- Kohli DR; Li Y; Khasabov SG; Gupta P; Kehl LJ; Ericson ME; Nguyen J; Gupta V; Hebbel RP; Simone DA; Gupta K. 2010. Pain-related behaviors and neurochemical alterations in mice expressing sickle hemoglobin: modulation by cannabinoids. Blood 116(3):456-65. PubMed: 20304807MGI: J:162824
- Lagoda G; Sezen SF; Hurt KJ; Cabrini MR; Mohanty DK; Burnett AL. 2014. Sustained nitric oxide (NO)-releasing compound reverses dysregulated NO signal transduction in priapism. FASEB J 28(1):76-84. PubMed: 24076963MGI: J:206623
- Lebensburger JD; Howard T; Hu Y; Pestina TI; Gao G; Johnson M; Zakharenko SS; Ware RE; Tuomanen EI; Persons DA; Rosch JW. 2012. Hydroxyurea therapy of a murine model of sickle cell anemia inhibits the progression of pneumococcal disease by down-modulating E-selectin. Blood 119(8):1915-21. PubMed: 22130804MGI: J:181749
- Levasseur DN; Ryan TM; Pawlik KM; Townes TM. 2003. Correction of a mouse model of sickle cell disease: lentiviral/antisickling beta-globin gene transduction of unmobilized, purified hematopoietic stem cells. Blood 102(13):4312-9. PubMed: 12933581MGI: J:134982
- Li J; Kim K; Hahm E; Molokie R; Hay N; Gordeuk VR; Du X; Cho J. 2014. Neutrophil AKT2 regulates heterotypic cell-cell interactions during vascular inflammation. J Clin Invest 124(4):1483-96. PubMed: 24642468MGI: J:209590
- Liu S; McConnell SC; Ryan TM. 2013. Erythropoiesis in the absence of adult hemoglobin. Mol Cell Biol 33(11):2241-51. PubMed: 23530053MGI: J:204029
- Manci EA; Hillery CA; Bodian CA; Zhang ZG; Lutty GA; Coller BS. 2006. Pathology of Berkeley sickle cell mice: similarities and differences with human sickle cell disease. Blood 107(4):1651-8. PubMed: 16166585MGI: J:129356
- McConnell SC; Huo Y; Liu S; Ryan TM. 2011. Human globin knock-in mice complete fetal-to-adult hemoglobin switching in postnatal development. Mol Cell Biol 31(4):876-83. PubMed: 21173165MGI: J:170380
- Meiler SE; Wade M; Kutlar F; Yerigenahally SD; Xue Y; Moutouh-de Parseval LA; Corral LG; Swerdlow PS; Kutlar A. 2011. Pomalidomide augments fetal hemoglobin production without the myelosuppressive effects of hydroxyurea in transgenic sickle cell mice. Blood 118(4):1109-12. PubMed: 21536862MGI: J:174901
- Mi T; Abbasi S; Zhang H; Uray K; Chunn JL; Xia LW; Molina JG; Weisbrodt NW; Kellems RE; Blackburn MR; Xia Y. 2008. Excess adenosine in murine penile erectile tissues contributes to priapism via A2B adenosine receptor signaling. J Clin Invest 118(4):1491-501. PubMed: 18340377MGI: J:135978
- Musicki B; Zhang Y; Chen H; Brown TR; Zirkin BR; Burnett AL. 2015. Mechanism of testosterone deficiency in the transgenic sickle cell mouse. PLoS One 10(5):e0128694. PubMed: 26023917MGI: J:237731
- Nandedkar SD; Feroah TR; Hutchins W; Weihrauch D; Konduri KS; Wang J; Strunk RC; DeBaun MR; Hillery CA; Pritchard KA. 2008. Histopathology of experimentally induced asthma in a murine model of sickle cell disease. Blood 112(6):2529-38. PubMed: 18579795MGI: J:138890
- Ning C; Wen J; Zhang Y; Dai Y; Wang W; Zhang W; Qi L; Grenz A; Eltzschig HK; Blackburn MR; Kellems RE; Xia Y. 2014. Excess adenosine A2B receptor signaling contributes to priapism through HIF-1alpha mediated reduction of PDE5 gene expression. FASEB J 28(6):2725-35. PubMed: 24614760MGI: J:216540
- Noguchi CT; Gladwin M; Diwan B; Merciris P; Smith R; Yu X; Buzard G; Fitzhugh A; Keefer LK; Schechter AN; Mohandas N. 2001. Pathophysiology of a sickle cell trait mouse model: human alpha(beta)(S) transgenes with one mouse beta-globin allele. Blood Cells Mol Dis 27(6):971-7. PubMed: 11831863MGI: J:128124
- Peranteau WH; Hayashi S; Abdulmalik O; Chen Q; Merchant A; Asakura T; Flake AW. 2015. Correction of murine hemoglobinopathies by prenatal tolerance induction and postnatal nonmyeloablative allogeneic BM transplants. Blood 126(10):1245-54. PubMed: 26124498MGI: J:226447
- Prado GN; Romero JR; Rivera A. 2013. Endothelin-1 receptor antagonists regulate cell surface-associated protein disulfide isomerase in sickle cell disease. FASEB J 27(11):4619-29. PubMed: 23913858MGI: J:203687
- Pritchard KA Jr; Feroah TR; Nandedkar SD; Holzhauer SL; Hutchins W; Schulte ML; Strunk RC; Debaun MR; Hillery CA. 2012. Effects of experimental asthma on inflammation and lung mechanics in sickle cell mice. Am J Respir Cell Mol Biol 46(3):389-96. PubMed: 22033263MGI: J:194579
- Pritchard KA Jr; Ou J; Ou Z; Shi Y; Franciosi JP; Signorino P; Kaul S; Ackland-Berglund C; Witte K; Holzhauer S; Mohandas N; Guice KS; Oldham KT; Hillery CA. 2004. Hypoxia-induced acute lung injury in murine models of sickle cell disease. Am J Physiol Lung Cell Mol Physiol 286(4):L705-14. PubMed: 12972407MGI: J:134696
- Ramos P; Casu C; Gardenghi S; Breda L; Crielaard BJ; Guy E; Marongiu MF; Gupta R; Levine RL; Abdel-Wahab O; Ebert BL; Van Rooijen N; Ghaffari S; Grady RW; Giardina PJ; Rivella S. 2013. Macrophages support pathological erythropoiesis in polycythemia vera and beta-thalassemia. Nat Med 19(4):437-45. PubMed: 23502961MGI: J:197988
- Romero JR; Suzuka SM; Nagel RL; Fabry ME. 2004. Expression of HbC and HbS, but not HbA, results in activation of K-Cl cotransport activity in transgenic mouse red cells. Blood 103(6):2384-90. PubMed: 14615383MGI: J:88563
- Ryan TM; Ciavatta DJ; Townes TM. 1997. Knockout-transgenic mouse model of sickle cell disease [see comments] Science 278(5339):873-6. PubMed: 9346487MGI: J:44160
- Solovey A; Kollander R; Shet A; Milbauer LC; Choong S; Panoskaltsis-Mortari A; Blazar BR; Kelm RJ Jr; Hebbel RP. 2004. Endothelial cell expression of tissue factor in sickle mice is augmented by hypoxia/reoxygenation and inhibited by lovastatin. Blood 104(3):840-6. PubMed: 15073034MGI: J:92287
- Sparkenbaugh EM; Chantrathammachart P; Chandarajoti K; Mackman N; Key NS; Pawlinski R. 2016. Thrombin-independent contribution of tissue factor to inflammation and cardiac hypertrophy in a mouse model of sickle cell disease. Blood 127(10):1371-3. PubMed: 26817955MGI: J:232634
- Sparkenbaugh EM; Chantrathammachart P; Mickelson J; van Ryn J; Hebbel RP; Monroe DM; Mackman N; Key NS; Pawlinski R. 2014. Differential contribution of FXa and thrombin to vascular inflammation in a mouse model of sickle cell disease. Blood 123(11):1747-56. PubMed: 24449213MGI: J:209655
- Sun K; Zhang Y; Bogdanov MV; Wu H; Song A; Li J; Dowhan W; Idowu M; Juneja HS; Molina JG; Blackburn MR; Kellems RE; Xia Y. 2015. Elevated adenosine signaling via adenosine A2B receptor induces normal and sickle erythrocyte sphingosine kinase 1 activity. Blood 125(10):1643-52. PubMed: 25587035MGI: J:221918
- Sundaram N; Tailor A; Mendelsohn L; Wansapura J; Wang X; Higashimoto T; Pauciulo MW; Gottliebson W; Kalra VK; Nichols WC; Kato GJ; Malik P. 2010. High levels of placenta growth factor in sickle cell disease promote pulmonary hypertension. Blood 116(1):109-12. PubMed: 20335221MGI: J:162823
- Szuber N; Buss JL; Soe-Lin S; Felfly H; Trudel M; Ponka P. 2008. Alternative treatment paradigm for thalassemia using iron chelators. Exp Hematol 36(7):773-85. PubMed: 18456387MGI: J:136991
- Vincent L; Vang D; Nguyen J; Gupta M; Luk K; Ericson ME; Simone DA; Gupta K. 2013. Mast cell activation contributes to sickle cell pathobiology and pain in mice. Blood 122(11):1853-62. PubMed: 23775718MGI: J:202290
- Wang L; Almeida LE; de Souza Batista CM; Khaibullina A; Xu N; Albani S; Guth KA; Seo JS; Quezado M; Quezado ZM. 2016. Cognitive and behavior deficits in sickle cell mice are associated with profound neuropathologic changes in hippocampus and cerebellum. Neurobiol Dis 85:60-72. PubMed: 26462816MGI: J:228649
- Wen J; Jiang X; Dai Y; Zhang Y; Tang Y; Sun H; Mi T; Phatarpekar PV; Kellems RE; Blackburn MR; Xia Y. 2010. Increased adenosine contributes to penile fibrosis, a dangerous feature of priapism, via A2B adenosine receptor signaling. FASEB J 24(3):740-9. PubMed: 19858092MGI: J:158034
- Wood KC; Hebbel RP; Granger DN. 2005. Endothelial cell NADPH oxidase mediates the cerebral microvascular dysfunction in sickle cell transgenic mice. FASEB J 19(8):989-91. PubMed: 15923406MGI: J:128246
- Wood KC; Hebbel RP; Granger DN. 2004. Endothelial cell P-selectin mediates a proinflammatory and prothrombogenic phenotype in cerebral venules of sickle cell transgenic mice. Am J Physiol Heart Circ Physiol 286(5):H1608-14. PubMed: 14704223MGI: J:95603
- Xu J; Peng C; Sankaran VG; Shao Z; Esrick EB; Chong BG; Ippolito GC; Fujiwara Y; Ebert BL; Tucker PW; Orkin SH. 2011. Correction of sickle cell disease in adult mice by interference with fetal hemoglobin silencing. Science 334(6058):993-6. PubMed: 21998251MGI: J:177861
- Zhang Y; Dai Y; Wen J; Zhang W; Grenz A; Sun H; Tao L; Lu G; Alexander DC; Milburn MV; Carter-Dawson L; Lewis DE; Zhang W; Eltzschig HK; Kellems RE; Blackburn MR; Juneja HS; Xia Y. 2011. Detrimental effects of adenosine signaling in sickle cell disease. Nat Med 17(1):79-86. PubMed: 21170046MGI: J:168473
- Zhao Y; Schwartz EA; Palmer GM; Zennadi R. 2016. MEK1/2 inhibitors reverse acute vascular occlusion in mouse models of sickle cell disease. FASEB J 30(3):1171-86. PubMed: 26631480MGI: J:230900
- de Jong K; Emerson RK; Butler J; Bastacky J; Mohandas N; Kuypers FA. 2001. Short survival of phosphatidylserine-exposing red blood cells in murine sickle cell anemia. Blood 98(5):1577-84. PubMed: 11520810MGI: J:131515
Additional - Tg(HBA-HBBs)41Paz related
- Archer DR; Stiles JK; Newman GW; Quarshie A; Hsu LL; Sayavongsa P; Perry J; Jackson EM; Hibbert JM. 2008. C-reactive protein and interleukin-6 are decreased in transgenic sickle cell mice fed a high protein diet. J Nutr 138(6):1148-52. PubMed: 18492848MGI: J:136372
- Arumugam PI; Mullins ES; Shanmukhappa SK; Monia BP; Loberg A; Shaw MA; Rizvi T; Wansapura J; Degen JL; Malik P. 2015. Genetic diminution of circulating prothrombin ameliorates multiorgan pathologies in sickle cell disease mice. Blood 126(15):1844-55. PubMed: 26286849MGI: J:230254
- Bakeer N; James J; Roy S; Wansapura J; Shanmukhappa SK; Lorenz JN; Osinska H; Backer K; Huby AC; Shrestha A; Niss O; Fleck R; Quinn CT; Taylor MD; Purevjav E; Aronow BJ; Towbin JA; Malik P. 2016. Sickle cell anemia mice develop a unique cardiomyopathy with restrictive physiology. Proc Natl Acad Sci U S A 113(35):E5182-91. PubMed: 27503873MGI: J:235608
- Barazia A; Li J; Kim K; Shabrani N; Cho J. 2015. Hydroxyurea with AKT2 inhibition decreases vaso-occlusive events in sickle cell disease mice. Blood 126(22):2511-7. PubMed: 26265698MGI: J:230085
- Barinaga M. 1997. Mutant mice mimic human sickle cell anemia [news; comment] Science 278(5339):803-4. PubMed: 9381190MGI: J:44159
- Belcher JD; Bryant CJ; Nguyen J; Bowlin PR; Kielbik MC; Bischof JC; Hebbel RP; Vercellotti GM. 2003. Transgenic sickle mice have vascular inflammation. Blood 101(10):3953-9. PubMed: 12543857MGI: J:83445
- Belcher JD; Mahaseth H; Welch TE; Otterbein LE; Hebbel RP; Vercellotti GM. 2006. Heme oxygenase-1 is a modulator of inflammation and vaso-occlusion in transgenic sickle mice. J Clin Invest 116(3):808-16. PubMed: 16485041MGI: J:106486
- Bivalacqua TJ; Musicki B; Hsu LL; Berkowitz DE; Champion HC; Burnett AL. 2013. Sildenafil citrate-restored eNOS and PDE5 regulation in sickle cell mouse penis prevents priapism via control of oxidative/nitrosative stress. PLoS One 8(7):e68028. PubMed: 23844149MGI: J:204301
- Champion HC; Bivalacqua TJ; Takimoto E; Kass DA; Burnett AL. 2005. Phosphodiesterase-5A dysregulation in penile erectile tissue is a mechanism of priapism. Proc Natl Acad Sci U S A 102(5):1661-6. PubMed: 15668387MGI: J:96103
- Chang J; Patton JT; Sarkar A; Ernst B; Magnani JL; Frenette PS. 2010. GMI-1070, a novel pan-selectin antagonist, reverses acute vascular occlusions in sickle cell mice. Blood 116(10):1779-86. PubMed: 20508165MGI: J:164535
- Chang JC; Ye L; Kan YW. 2006. Correction of the sickle cell mutation in embryonic stem cells. Proc Natl Acad Sci U S A 103(4):1036-40. PubMed: 16407095MGI: J:105707
- Chantrathammachart P; Mackman N; Sparkenbaugh E; Wang JG; Parise LV; Kirchhofer D; Key NS; Pawlinski R. 2012. Tissue factor promotes activation of coagulation and inflammation in a mouse model of sickle cell disease. Blood 120(3):636-46. PubMed: 22661702MGI: J:189104
- Chen G; Zhang D; Fuchs TA; Manwani D; Wagner DD; Frenette PS. 2014. Heme-induced neutrophil extracellular traps contribute to the pathogenesis of sickle cell disease. Blood 123(24):3818-27. PubMed: 24620350MGI: J:210886
- Dasgupta T; Fabry ME; Kaul DK. 2010. Antisickling property of fetal hemoglobin enhances nitric oxide bioavailability and ameliorates organ oxidative stress in transgenic-knockout sickle mice. Am J Physiol Regul Integr Comp Physiol 298(2):R394-402. PubMed: 20007516MGI: J:156179
- Dasgupta T; Hebbel RP; Kaul DK. 2006. Protective effect of arginine on oxidative stress in transgenic sickle mouse models. Free Radic Biol Med 41(12):1771-80. PubMed: 17157180MGI: J:116710
- Eiymo Mwa Mpollo MS; Brandt EB; Shanmukhappa SK; Arumugam PI; Tiwari S; Loberg A; Pillis D; Rizvi T; Lindsey M; Jonck B; Carmeliet P; Kalra VK; Le Cras TD; Ratner N; Wills-Karp M; Hershey GK; Malik P. 2016. Placenta growth factor augments airway hyperresponsiveness via leukotrienes and IL-13. J Clin Invest 126(2):571-84. PubMed: 26690703MGI: J:231182
- Gavins FN; Russell J; Senchenkova EL; De Almeida Paula L; Damazo AS; Esmon CT; Kirchhofer D; Hebbel RP; Granger DN. 2011. Mechanisms of enhanced thrombus formation in cerebral microvessels of mice expressing hemoglobin-S. Blood 117(15):4125-33. PubMed: 21304105MGI: J:172840
- Gutsaeva DR; Montero-Huerta P; Parkerson JB; Yerigenahally SD; Ikuta T; Head CA. 2014. Molecular mechanisms underlying synergistic adhesion of sickle red blood cells by hypoxia and low nitric oxide bioavailability. Blood 123(12):1917-26. PubMed: 24429338MGI: J:209498
- Gutsaeva DR; Parkerson JB; Yerigenahally SD; Kurz JC; Schaub RG; Ikuta T; Head CA. 2011. Inhibition of cell adhesion by anti-P-selectin aptamer: a new potential therapeutic agent for sickle cell disease. Blood 117(2):727-35. PubMed: 20926770MGI: J:168427
- He Z; Russell JE. 2004. Antisickling effects of an endogenous human alpha-like globin. Nat Med 10(4):365-7. PubMed: 15034572MGI: J:131270
- Hidalgo A; Chang J; Jang JE; Peired AJ; Chiang EY; Frenette PS. 2009. Heterotypic interactions enabled by polarized neutrophil microdomains mediate thromboinflammatory injury. Nat Med 15(4):384-91. PubMed: 19305412MGI: J:149370
- Holtzclaw JD; Jack D; Aguayo SM; Eckman JR; Roman J; Hsu LL. 2004. Enhanced pulmonary and systemic response to endotoxin in transgenic sickle mice. Am J Respir Crit Care Med 169(6):687-95. PubMed: 14684557MGI: J:132062
- Hsu LL; Champion HC; Campbell-Lee SA; Bivalacqua TJ; Manci EA; Diwan BA; Schimel DM; Cochard AE; Wang X; Schechter AN; Noguchi CT; Gladwin MT. 2007. Hemolysis in sickle cell mice causes pulmonary hypertension due to global impairment in nitric oxide bioavailability. Blood 109(7):3088-98. PubMed: 17158223MGI: J:145347
- Ieremia J; Blau CA. 2002. Limitations of a mouse model of sickle cell anemia. Blood Cells Mol Dis 28(2):146-51. PubMed: 12064910MGI: J:128113
- Jang JE; Hidalgo A; Frenette PS. 2012. Intravenous immunoglobulins modulate neutrophil activation and vascular injury through FcgammaRIII and SHP-1. Circ Res 110(8):1057-66. PubMed: 22415018MGI: J:212521
- Kaul DK; Fabry ME; Suzuka SM; Zhang X. 2013. Antisickling fetal hemoglobin reduces hypoxia-inducible factor-1alpha expression in normoxic sickle mice: microvascular implications. Am J Physiol Heart Circ Physiol 304(1):H42-50. PubMed: 23125209MGI: J:192836
- Kaul DK; Liu XD; Chang HY; Nagel RL; Fabry ME. 2004. Effect of fetal hemoglobin on microvascular regulation in sickle transgenic-knockout mice. J Clin Invest 114(8):1136-45. PubMed: 15489961MGI: J:93424
- Kiefmann R; Rifkind JM; Nagababu E; Bhattacharya J. 2008. Red blood cells induce hypoxic lung inflammation. Blood 111(10):5205-14. PubMed: 18270324MGI: J:135569
- Kohli DR; Li Y; Khasabov SG; Gupta P; Kehl LJ; Ericson ME; Nguyen J; Gupta V; Hebbel RP; Simone DA; Gupta K. 2010. Pain-related behaviors and neurochemical alterations in mice expressing sickle hemoglobin: modulation by cannabinoids. Blood 116(3):456-65. PubMed: 20304807MGI: J:162824
- Lagoda G; Sezen SF; Hurt KJ; Cabrini MR; Mohanty DK; Burnett AL. 2014. Sustained nitric oxide (NO)-releasing compound reverses dysregulated NO signal transduction in priapism. FASEB J 28(1):76-84. PubMed: 24076963MGI: J:206623
- Lebensburger JD; Howard T; Hu Y; Pestina TI; Gao G; Johnson M; Zakharenko SS; Ware RE; Tuomanen EI; Persons DA; Rosch JW. 2012. Hydroxyurea therapy of a murine model of sickle cell anemia inhibits the progression of pneumococcal disease by down-modulating E-selectin. Blood 119(8):1915-21. PubMed: 22130804MGI: J:181749
- Levasseur DN; Ryan TM; Pawlik KM; Townes TM. 2003. Correction of a mouse model of sickle cell disease: lentiviral/antisickling beta-globin gene transduction of unmobilized, purified hematopoietic stem cells. Blood 102(13):4312-9. PubMed: 12933581MGI: J:134982
- Li J; Kim K; Hahm E; Molokie R; Hay N; Gordeuk VR; Du X; Cho J. 2014. Neutrophil AKT2 regulates heterotypic cell-cell interactions during vascular inflammation. J Clin Invest 124(4):1483-96. PubMed: 24642468MGI: J:209590
- Manci EA; Hillery CA; Bodian CA; Zhang ZG; Lutty GA; Coller BS. 2006. Pathology of Berkeley sickle cell mice: similarities and differences with human sickle cell disease. Blood 107(4):1651-8. PubMed: 16166585MGI: J:129356
- Meiler SE; Wade M; Kutlar F; Yerigenahally SD; Xue Y; Moutouh-de Parseval LA; Corral LG; Swerdlow PS; Kutlar A. 2011. Pomalidomide augments fetal hemoglobin production without the myelosuppressive effects of hydroxyurea in transgenic sickle cell mice. Blood 118(4):1109-12. PubMed: 21536862MGI: J:174901
- Mi T; Abbasi S; Zhang H; Uray K; Chunn JL; Xia LW; Molina JG; Weisbrodt NW; Kellems RE; Blackburn MR; Xia Y. 2008. Excess adenosine in murine penile erectile tissues contributes to priapism via A2B adenosine receptor signaling. J Clin Invest 118(4):1491-501. PubMed: 18340377MGI: J:135978
- Musicki B; Zhang Y; Chen H; Brown TR; Zirkin BR; Burnett AL. 2015. Mechanism of testosterone deficiency in the transgenic sickle cell mouse. PLoS One 10(5):e0128694. PubMed: 26023917MGI: J:237731
- Nandedkar SD; Feroah TR; Hutchins W; Weihrauch D; Konduri KS; Wang J; Strunk RC; DeBaun MR; Hillery CA; Pritchard KA. 2008. Histopathology of experimentally induced asthma in a murine model of sickle cell disease. Blood 112(6):2529-38. PubMed: 18579795MGI: J:138890
- Ning C; Wen J; Zhang Y; Dai Y; Wang W; Zhang W; Qi L; Grenz A; Eltzschig HK; Blackburn MR; Kellems RE; Xia Y. 2014. Excess adenosine A2B receptor signaling contributes to priapism through HIF-1alpha mediated reduction of PDE5 gene expression. FASEB J 28(6):2725-35. PubMed: 24614760MGI: J:216540
- Noguchi CT; Gladwin M; Diwan B; Merciris P; Smith R; Yu X; Buzard G; Fitzhugh A; Keefer LK; Schechter AN; Mohandas N. 2001. Pathophysiology of a sickle cell trait mouse model: human alpha(beta)(S) transgenes with one mouse beta-globin allele. Blood Cells Mol Dis 27(6):971-7. PubMed: 11831863MGI: J:128124
- Prado GN; Romero JR; Rivera A. 2013. Endothelin-1 receptor antagonists regulate cell surface-associated protein disulfide isomerase in sickle cell disease. FASEB J 27(11):4619-29. PubMed: 23913858MGI: J:203687
- Pritchard KA Jr; Feroah TR; Nandedkar SD; Holzhauer SL; Hutchins W; Schulte ML; Strunk RC; Debaun MR; Hillery CA. 2012. Effects of experimental asthma on inflammation and lung mechanics in sickle cell mice. Am J Respir Cell Mol Biol 46(3):389-96. PubMed: 22033263MGI: J:194579
- Pritchard KA Jr; Ou J; Ou Z; Shi Y; Franciosi JP; Signorino P; Kaul S; Ackland-Berglund C; Witte K; Holzhauer S; Mohandas N; Guice KS; Oldham KT; Hillery CA. 2004. Hypoxia-induced acute lung injury in murine models of sickle cell disease. Am J Physiol Lung Cell Mol Physiol 286(4):L705-14. PubMed: 12972407MGI: J:134696
- Ryan TM; Ciavatta DJ; Townes TM. 1997. Knockout-transgenic mouse model of sickle cell disease [see comments] Science 278(5339):873-6. PubMed: 9346487MGI: J:44160
- Sparkenbaugh EM; Chantrathammachart P; Chandarajoti K; Mackman N; Key NS; Pawlinski R. 2016. Thrombin-independent contribution of tissue factor to inflammation and cardiac hypertrophy in a mouse model of sickle cell disease. Blood 127(10):1371-3. PubMed: 26817955MGI: J:232634
- Sparkenbaugh EM; Chantrathammachart P; Mickelson J; van Ryn J; Hebbel RP; Monroe DM; Mackman N; Key NS; Pawlinski R. 2014. Differential contribution of FXa and thrombin to vascular inflammation in a mouse model of sickle cell disease. Blood 123(11):1747-56. PubMed: 24449213MGI: J:209655
- Sun K; Zhang Y; Bogdanov MV; Wu H; Song A; Li J; Dowhan W; Idowu M; Juneja HS; Molina JG; Blackburn MR; Kellems RE; Xia Y. 2015. Elevated adenosine signaling via adenosine A2B receptor induces normal and sickle erythrocyte sphingosine kinase 1 activity. Blood 125(10):1643-52. PubMed: 25587035MGI: J:221918
- Sundaram N; Tailor A; Mendelsohn L; Wansapura J; Wang X; Higashimoto T; Pauciulo MW; Gottliebson W; Kalra VK; Nichols WC; Kato GJ; Malik P. 2010. High levels of placenta growth factor in sickle cell disease promote pulmonary hypertension. Blood 116(1):109-12. PubMed: 20335221MGI: J:162823
- Wang L; Almeida LE; de Souza Batista CM; Khaibullina A; Xu N; Albani S; Guth KA; Seo JS; Quezado M; Quezado ZM. 2016. Cognitive and behavior deficits in sickle cell mice are associated with profound neuropathologic changes in hippocampus and cerebellum. Neurobiol Dis 85:60-72. PubMed: 26462816MGI: J:228649
- Wen J; Jiang X; Dai Y; Zhang Y; Tang Y; Sun H; Mi T; Phatarpekar PV; Kellems RE; Blackburn MR; Xia Y. 2010. Increased adenosine contributes to penile fibrosis, a dangerous feature of priapism, via A2B adenosine receptor signaling. FASEB J 24(3):740-9. PubMed: 19858092MGI: J:158034
- Wood KC; Hebbel RP; Granger DN. 2004. Endothelial cell P-selectin mediates a proinflammatory and prothrombogenic phenotype in cerebral venules of sickle cell transgenic mice. Am J Physiol Heart Circ Physiol 286(5):H1608-14. PubMed: 14704223MGI: J:95603
- Wood KC; Hebbel RP; Granger DN. 2005. Endothelial cell NADPH oxidase mediates the cerebral microvascular dysfunction in sickle cell transgenic mice. FASEB J 19(8):989-91. PubMed: 15923406MGI: J:128246
- Xu J; Peng C; Sankaran VG; Shao Z; Esrick EB; Chong BG; Ippolito GC; Fujiwara Y; Ebert BL; Tucker PW; Orkin SH. 2011. Correction of sickle cell disease in adult mice by interference with fetal hemoglobin silencing. Science 334(6058):993-6. PubMed: 21998251MGI: J:177861
- Zhang Y; Dai Y; Wen J; Zhang W; Grenz A; Sun H; Tao L; Lu G; Alexander DC; Milburn MV; Carter-Dawson L; Lewis DE; Zhang W; Eltzschig HK; Kellems RE; Blackburn MR; Juneja HS; Xia Y. 2011. Detrimental effects of adenosine signaling in sickle cell disease. Nat Med 17(1):79-86. PubMed: 21170046MGI: J:168473
- Zhao Y; Schwartz EA; Palmer GM; Zennadi R. 2016. MEK1/2 inhibitors reverse acute vascular occlusion in mouse models of sickle cell disease. FASEB J 30(3):1171-86. PubMed: 26631480MGI: J:230900
- de Jong K; Emerson RK; Butler J; Bastacky J; Mohandas N; Kuypers FA. 2001. Short survival of phosphatidylserine-exposing red blood cells in murine sickle cell anemia. Blood 98(5):1577-84. PubMed: 11520810MGI: J:131515