JAX
Mouse Strain Datasheet - 017639
STOCK Nf1tm1Par/J
Also Known As: Nf1flox
These Nf1flox mice possess loxP sites flanking exons 31-32 of the neurofibromatosis 1 gene (Nf1), and have applications in studying cancer, neural crest development and neurofibromatosis type I.
Donating Investigator
Luis F Parada, UT Southwestern Medical Center
Genetic overview
| Genetic Background | Generation |
|---|---|
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Nf1tm1Par
| Allele Type | Gene Symbol | Gene Name |
|---|---|---|
| Targeted (Conditional ready (e.g. floxed), No functional change) | Nf1 | neurofibromin 1 |
Research Applications
- Cancer Research
- Neurobiology Research
- Research Tools
Detailed Description
Mutation in the human neurofibromin gene, NF1, is the cause of the autosomal dominant disorder Type I Neurofibromatosis. These mice possess loxP sites on either side of exons 31 and 32 of the targeted gene. Mice that are homozygous for this allele are viable, fertile, normal in size and do not display any gross physical or behavioral abnormalities. When these mutant mice are bred to mice that express Cre recombinase, resulting offspring will have exons 31 and 32 deleted in the cre-expressing tissue(s).
When bred to a strain with Cre recombinase expression in the developing neural tube (see Stock No. 009107 for example), this mutant mouse strain may be useful in studies of Type I Neurofibromatosis.
When bred to a strain with Cre recombinase expression in neuronal cells (see Stock No. 003966 for example), this mutant mouse strain may be useful in studies of cerebral cortex development and reactive astrogliosis.
When bred to a strain with Cre recombinase expression in endothelial cells (see Stock No. 008863 for example), this mutant mouse strain may be useful in studies of neural crest development.
When bred to B6.Cg-Tg(Prrx1-cre)1Cjt/J mice (Stock No. 005584), mesenchyme-specific cre-expression results in mice that exhibit an increase in the amount of connective tissue, as well as muscle dystrophy characterized by fibrosis, a reduced number of muscle fibers, and reduced muscle force.
When bred to mice carrying Tg(Mx1-cre)1Cgn (Stock No. 003556), interferon-induced Cre-mediated recombination results in a progressive myeloproliferative disorder.
This allele is also part of the MADM-TG,p53KO,NF1-flox strain (Stock No. 017530), which is a genetic mosaicism model for cancer.
In an attempt to offer alleles on well-characterized or multiple genetic backgrounds, alleles are frequently moved to a genetic background different from that on which an allele was first characterized. This is the case for the strain above. It should be noted that the phenotype could vary from that originally described. We will modify the strain description if necessary as published results become available.
Development
A targeting vector containing a loxP site and a PGKNeo cassette was inserted upstream of exon 31. A second loxP site was inserted downstream of exon 32. The construct was electroporated into 129 derived R1 embryonic stem (ES) cells. Correctly targeted ES cells were injected into C57BL/6 blastocysts. The resulting chimeric animals were crossed to C57BL/6 mice. The mice were then crossed to several other mutant lines. The combination mutant strain, with a mix of CD1, C57BL/6 and 129 genetic backgrounds, was crossed to C57BL/6J to separate the Nf1tm1Par allele.
Control Suggestions
Approximate Controls
Additional Information
Selected References
- Liu C; Sage JC; Miller MR; Verhaak RG; Hippenmeyer S; Vogel H; Foreman O; Bronson RT; Nishiyama A; Luo L; Zong H. 2011. Mosaic analysis with double markers reveals tumor cell of origin in glioma. Cell 146(2):209-21. PubMed: 21737130MGI: J:174616
- Zhu Y; Romero MI; Ghosh P; Ye Z; Charnay P; Rushing EJ; Marth JD; Parada LF. 2001. Ablation of NF1 function in neurons induces abnormal development of cerebral cortex and reactive gliosis in the brain. Genes Dev 15(7):859-76. PubMed: 11297510MGI: J:68558
Nf1tm1Par
Allele Symbol: Nf1tm1Par
| Allele Name | targeted mutation 1, Luis F Parada |
|---|---|
| Allele Type | Targeted (Conditional ready (e.g. floxed), No functional change) |
| Allele Synonym(s) | Nf1flox; Nf1flox |
| Gene Symbol and Name | Nf1, neurofibromin 1 |
| Gene Synonym(s) | AW494271; Dsk9; Dsk9; Martin Hrabe de Angelis dark skin 9; Mhdadsk9; Mhdadsk9; NFNS; Nf-1; Nf-1; VRNF; WSS; dark skin 9; expressed sequence AW494271; neurofibromatosis 1; neurofibromin |
| Site of Expression | When mice carrying this allele are mated to a Synapsin I promoter driven Cre transgenic mouse strain, NF1 function is abated in most differentiated neuronal populations, resulting in abnormal development of the cerebral cortex. |
| Strain of Origin | (129X1/SvJ x 129S1/Sv)F1-Kitl<+> |
| Chromosome | 11 |
| Molecular Note | A loxP-neomycin selection cassette was inserted into intron 30 and a single loxP site was inserted into intron 32. |
| Mutations Made By | Steven McKinnon, UT Southwestern Medical Center |
Disease Terms
Research Areas By Genotype
This mouse can be used to support research in many areas including:
- Cancer Research
- Increased Tumor Incidence
- Other Tissues/Organs: brain
- Increased Tumor Incidence
- Neurobiology Research
- Cre-lox System
- loxP-flanked Sequences
- Cre-lox System
- Research Tools
- Cre-lox System
- loxP-flanked Sequences
- Cre-lox System
Mammalian Phenotype Terms by Genotype
The following phenotype information is associated with a similar, but not exact match to this JAX® Mice strain
Genotype: Nf1tm1Par/Nf1tm1Par
involves: 129S1/Sv * 129X1/SvJ
nervous system phenotype
- abnormal astrocyte physiology
- astrocytes transfected with a cre-expresing adenovirus exhibit increased proliferation compared to in control cells (MGI Ref ID J:176586)
The following phenotype relates to a compound genotype created using this strain
Genotype: Nf1tm1Par/Nf1tm1Par Tg(Gfap-cre)77.6Mvs/0
involves: 129S1/Sv * 129X1/SvJ * BALB/c * C57BL/6NHsd
neoplasm
- neoplasm
- mutants do not develop tumors (MGI Ref ID J:154673)
Genotype: Nf1tm1Par/Nf1tm1Par Tg(Mx1-cre)1Cgn/0
involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * CBA
mortality/aging
- premature death
- 50% of pIpC treated mice die by 7.5 months of age (MGI Ref ID J:90973)
hematopoietic system phenotype
- abnormal bone marrow cell morphology/development
- apoptosis is reduced in the bone marrow of pIpC injected mice (MGI Ref ID J:90973)
- bone marrow from pIpC injected mice contains elevated numbers of CFU-GM and an increase in the numbers of CFU-GM that are hypersensitive to granulocyte-macrophage colony stimulating factor (GM-CSF) and CFU-GM colonies are larger than normal and show abnormal spreading morphologymal spreading morphology (MGI Ref ID J:90973)
- bone marrow from pIpC injected mice is highly cellular, comprised of myeloid cells at various stages of differentiation (MGI Ref ID J:90973)
- increase in numbers of immature monocytic cells in the bone marrow of pIpC treated mice (MGI Ref ID J:90973)
- abnormal common myeloid progenitor cell morphology
- myeloid progenitors from pIpC injected mice show increased proliferation (MGI Ref ID J:90973)
- abnormal definitive hematopoiesis
- abnormal hematopoietic cell number
- pIpC injected mice at 3 to 5 days of age exhibit elevated numbers of differentiated lymphoid cells by 3 months of age (MGI Ref ID J:90973)
- abnormal myeloid leukocyte morphology
- pIpC injected mice at 3 to 5 days of age exhibit elevated numbers of differentiated myeloid cells by 3 months of age (MGI Ref ID J:90973)
- abnormal myelopoiesis
- spleen from pIpC injected mice shows a massive increase in myelopoiesis (MGI Ref ID J:90973)
- abnormal spleen morphology
- spleens from pIpC injected mice contain large numbers of CFU-GM (MGI Ref ID J:90973)
- enlarged spleen
- pIpC injected mice at 3 to 5 days of age exhibit progressive splenomegaly with extensive infiltration of myeloid cells at various stages of maturation (MGI Ref ID J:90973)
- increased granulocyte number
- pIpC injected mice show an increase in numbers of differentiated granulocytic cells (MGI Ref ID J:90973)
- increased leukocyte cell number
- increased lymphocyte cell number
- pIpC injected mice at 3 to 5 days of age exhibit increased numbers of morphologically normal lymphocytes by 3 months of age (MGI Ref ID J:90973)
- increased monocyte cell number
- increased neutrophil cell number
- pIpC injected mice at 3 to 5 days of age exhibit increased numbers of morphologically normal neutrophils by 3 months of age (MGI Ref ID J:90973)
behavior/neurological phenotype
- abnormal gait
- pIpC injected mice at 3 to 5 days of age exhibit hunching by 5-6 months of age (MGI Ref ID J:90973)
- hunched posture
- pIpC injected mice at 3 to 5 days of age exhibit abnormal gait by 5-6 months of age (MGI Ref ID J:90973)
immune system phenotype
- abnormal myeloid leukocyte morphology
- pIpC injected mice at 3 to 5 days of age exhibit elevated numbers of differentiated myeloid cells by 3 months of age (MGI Ref ID J:90973)
- abnormal myelopoiesis
- spleen from pIpC injected mice shows a massive increase in myelopoiesis (MGI Ref ID J:90973)
- abnormal spleen morphology
- spleens from pIpC injected mice contain large numbers of CFU-GM (MGI Ref ID J:90973)
- enlarged spleen
- pIpC injected mice at 3 to 5 days of age exhibit progressive splenomegaly with extensive infiltration of myeloid cells at various stages of maturation (MGI Ref ID J:90973)
- increased granulocyte number
- pIpC injected mice show an increase in numbers of differentiated granulocytic cells (MGI Ref ID J:90973)
- increased leukocyte cell number
- increased lymphocyte cell number
- pIpC injected mice at 3 to 5 days of age exhibit increased numbers of morphologically normal lymphocytes by 3 months of age (MGI Ref ID J:90973)
- increased monocyte cell number
- increased neutrophil cell number
- pIpC injected mice at 3 to 5 days of age exhibit increased numbers of morphologically normal neutrophils by 3 months of age (MGI Ref ID J:90973)
integument phenotype
- disheveled coat
- pIpC injected mice at 3 to 5 days of age have a disheveled appearance by 5-6 months of age (MGI Ref ID J:90973)
Genotype: Nf1tm1Par/Nf1tm1Par Tg(Prrx1-cre)1Cjt/0
involves: 129S1/Sv * 129X1/SvJ * C57BL/6J * C57BL/6N * SJL/J
skeleton phenotype
- abnormal bone healing
- accumulation and persistence of fibrous tissue in the fracture gap, with increased numbers of osteoclasts (MGI Ref ID J:193350)
- ectopic fat tissue is seen in the fracture site (MGI Ref ID J:193350)
- increase in number of blood vessels in mutant fractures (in callus) compared to controls, indicating increased vascularization of the fracture tissue, however no osteogenesis results from this increased vascularization (MGI Ref ID J:193350)
- mutants exhibit delayed and defective fracture healing characterized by diminished cartilaginous callus formation, increased bone formation near the cortical bone on the periosteum but not in the fracture gap, and persistence of cartilage at day 21 such that bony bridging is not observedhat bony bridging is not observed (MGI Ref ID J:193350)
- while total callus volume following fracture is larger in mutants at day 7 and 10 due to rapid initial growth of fibrous tissue (desmal type ossification), by day 14 and 21, the total callus volume is significantly smaller (MGI Ref ID J:193350)
- abnormal cartilage morphology
- bone fractures exhibit impaired cartilage formation and increased periosteal ossification at the cortices (MGI Ref ID J:193350)
- abnormal endochondral bone ossification
- endochondrial formation is impaired following fracture but periosteal bone formation is enhanced (MGI Ref ID J:193350)
- abnormal osteoblast cell number
- fewer osteoblasts are seen within the fracture gap throughout healing compared to controls, however, osteoblast number is increased at the periosteal surface (MGI Ref ID J:193350)
- abnormal osteoid morphology
- presence of large areas of non-mineralized osteoid in the fracture gap, indicating decreased mineralization of the extracellular matrix (MGI Ref ID J:193350)
- decreased bone mineral density
- decrease of regenerative tissue bone mineral density following fracture (MGI Ref ID J:193350)
- fragile skeleton
- increased compact bone volume
- dramatic cortical bone thickening following fracture (MGI Ref ID J:193350)
- increased osteoclast cell number
- callus following fracture shows increased number of osteoclasts; most osteoclasts are localized within fibrous tissue and not on the bone surface as in controls (MGI Ref ID J:193350)
- increased osteoid thickness
- thickening of the osteoid layer in the periosteal region following fracture (MGI Ref ID J:193350)
homeostasis/metabolism phenotype
- abnormal bone healing
- accumulation and persistence of fibrous tissue in the fracture gap, with increased numbers of osteoclasts (MGI Ref ID J:193350)
- ectopic fat tissue is seen in the fracture site (MGI Ref ID J:193350)
- increase in number of blood vessels in mutant fractures (in callus) compared to controls, indicating increased vascularization of the fracture tissue, however no osteogenesis results from this increased vascularization (MGI Ref ID J:193350)
- mutants exhibit delayed and defective fracture healing characterized by diminished cartilaginous callus formation, increased bone formation near the cortical bone on the periosteum but not in the fracture gap, and persistence of cartilage at day 21 such that bony bridging is not observedhat bony bridging is not observed (MGI Ref ID J:193350)
- while total callus volume following fracture is larger in mutants at day 7 and 10 due to rapid initial growth of fibrous tissue (desmal type ossification), by day 14 and 21, the total callus volume is significantly smaller (MGI Ref ID J:193350)
hematopoietic system phenotype
- increased osteoclast cell number
- callus following fracture shows increased number of osteoclasts; most osteoclasts are localized within fibrous tissue and not on the bone surface as in controls (MGI Ref ID J:193350)
immune system phenotype
- increased osteoclast cell number
- callus following fracture shows increased number of osteoclasts; most osteoclasts are localized within fibrous tissue and not on the bone surface as in controls (MGI Ref ID J:193350)
Genotype: Nf1tm1Par/Nf1tm1Par Tg(Prrx1-cre)1Cjt/0
involves: 129S1/Sv * 129X1/SvJ * C57BL/6J * SJL/J
growth/size/body region phenotype
- decreased body weight
- weight is on average reduced by 25% (MGI Ref ID J:173779)
limbs/digits/tail phenotype
- short limbs
- short-limbed dwarfism, with mutants showing a reduction in entire limb size (MGI Ref ID J:173779)
muscle phenotype
- abnormal muscle development
- distal muscle groups in the extremities are most affected, with some muscles completely missing, indicating that the muscle differentiation process is disturbed (MGI Ref ID J:173779)
- marker analysis indicates a defect in muscle formation at E13.5, with specific muscle primordial reduced in size or entirely missing; approximate 30% reduction in the m. triceps size and about 50% reduction in the m. gluteus maximus size of E13.5 embryos (MGI Ref ID J:173779)
- the m. latissimus dorsi appears smaller and shows rarefaction of muscle fibers (MGI Ref ID J:173779)
- abnormal muscle fiber morphology
- abnormal muscle morphology
- large areas of dystrophic musculature are occupied by fat tissue (MGI Ref ID J:173779)
- muscle connective tissue shows increased proliferation at E14.5 and an increase in connective tissue in muscles is already seen at E16.5 (MGI Ref ID J:173779)
- muscle fibers are thinned out at E16.5 (MGI Ref ID J:173779)
- mutants exhibit muscle dystrophy (MGI Ref ID J:173779)
- abnormal muscle physiology
- abnormal myoblast differentiation
- abnormal myogenesis
- defect in myogenesis affecting the terminal differentiation of myoblasts between E12.5 and E14.5 (MGI Ref ID J:173779)
- decreased muscle weight
- weight of triceps muscle is reduced by more than 50% (MGI Ref ID J:173779)
- decreased skeletal muscle fiber number
- total number of muscle fibers is reduced by 50% in the triceps (MGI Ref ID J:173779)
- decreased skeletal muscle mass
- reduction in muscle size and mass (MGI Ref ID J:173779)
- skeletal muscle fibrosis
- generalized muscle fibrosis, characterized by expansion of collagen-rich connective tissue, and reduction in total number of muscle fibers (MGI Ref ID J:173779)
cellular phenotype
- abnormal myoblast differentiation
Genotype: Nf1tm1Par/Nf1tm1Par Tg(Syn1-cre)671Jxm/0
involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * CBA
growth/size/body region phenotype
- decreased body weight
- body weight and size are about 50% of normal (MGI Ref ID J:68558)
- postnatal growth retardation
- 3-4 days after birth, mice begin to exhibit growth retardation that is sustained into adulthood (MGI Ref ID J:68558)
nervous system phenotype
- abnormal cerebral cortex morphology
- increase in cell density in the cerebral cortex, often resulting in less apparent lamination (MGI Ref ID J:68558)
- astrocytosis
- mice display astrogliosis in various brain regions, however do not develop neuronal degeneration or microgliosis (MGI Ref ID J:68558)
- decreased forebrain size
- forebrain, but not the rest of the brain, is reduced in size, however mice exhibit normal neuronal development (MGI Ref ID J:68558)
- thin cerebral cortex
- about 20% reduction in coritcal thickness (MGI Ref ID J:68558)
behavior/neurological phenotype
- abnormal learning/memory/conditioning
- mice display severe learning disability (MGI Ref ID J:68558)
neoplasm
- neoplasm
- no evidence of tumors; optic gliomas, astrocytomas, or neurofibroma are not observed (MGI Ref ID J:68558)
Genotype: Nf1tm1Par/Nf1tm1Par Tg(Tek-cre)1Ywa/0
involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * SJL
cardiovascular system phenotype
- double outlet right ventricle
- seen in 8 of 11 embryos (MGI Ref ID J:80323)
- increased atrioventricular cushion size
- 8 of 11 embryos show an enlarged atrioventricular cushion (MGI Ref ID J:80323)
- pericardial effusion
- (MGI Ref ID J:80323)
- thin myocardium
- 8 of 11 embryos exhibit a thinned myocardium (MGI Ref ID J:80323)
- thin myocardium compact layer
- (MGI Ref ID J:80323)
- ventricular septal defect
- 10 of 11 embryos exhibit ventricular septal defects (MGI Ref ID J:80323)
homeostasis/metabolism phenotype
- pericardial effusion
- (MGI Ref ID J:80323)
References
- Liu C; Sage JC; Miller MR; Verhaak RG; Hippenmeyer S; Vogel H; Foreman O; Bronson RT; Nishiyama A; Luo L; Zong H. 2011. Mosaic analysis with double markers reveals tumor cell of origin in glioma. Cell 146(2):209-21. PubMed: 21737130MGI: J:174616
- Zhu Y; Romero MI; Ghosh P; Ye Z; Charnay P; Rushing EJ; Marth JD; Parada LF. 2001. Ablation of NF1 function in neurons induces abnormal development of cerebral cortex and reactive gliosis in the brain. Genes Dev 15(7):859-76. PubMed: 11297510MGI: J:68558
Additional - Nf1tm1Par related
- Alanne MH; Siljamaki E; Peltonen S; Vaananen K; Windle JJ; Parada LF; Maatta JA; Peltonen J. 2012. Phenotypic characterization of transgenic mice harboring Nf1+/- or Nf1-/- osteoclasts in otherwise Nf1+/+ background. J Cell Biochem 113(6):2136-46. PubMed: 22307743MGI: J:211765
- Alcantara Llaguno S; Chen J; Kwon CH; Jackson EL; Li Y; Burns DK; Alvarez-Buylla A; Parada LF. 2009. Malignant astrocytomas originate from neural stem/progenitor cells in a somatic tumor suppressor mouse model. Cancer Cell 15(1):45-56. PubMed: 19111880MGI: J:143505
- Anastasaki C; Woo AS; Messiaen LM; Gutmann DH. 2015. Elucidating the impact of neurofibromatosis-1 germline mutations on neurofibromin function and dopamine-based learning. Hum Mol Genet 24(12):3518-28. PubMed: 25788518MGI: J:221342
- Baek ST; Tallquist MD. 2012. Nf1 limits epicardial derivative expansion by regulating epithelial to mesenchymal transition and proliferation. Development 139(11):2040-9. PubMed: 22535408MGI: J:183991
- Bajenaru ML; Garbow JR; Perry A; Hernandez MR; Gutmann DH. 2005. Natural history of neurofibromatosis 1-associated optic nerve glioma in mice. Ann Neurol 57(1):119-27. PubMed: 15622533MGI: J:96293
- Bajenaru ML; Hernandez MR; Perry A; Zhu Y; Parada LF; Garbow JR; Gutmann DH. 2003. Optic nerve glioma in mice requires astrocyte Nf1 gene inactivation and Nf1 brain heterozygosity. Cancer Res 63(24):8573-7. PubMed: 14695164MGI: J:87063
- Bajenaru ML; Zhu Y; Hedrick NM; Donahoe J; Parada LF; Gutmann DH. 2002. Astrocyte-specific inactivation of the neurofibromatosis 1 gene (NF1) is insufficient for astrocytoma formation. Mol Cell Biol 22(14):5100-13. PubMed: 12077339MGI: J:77208
- Banerjee D; Hegedus B; Gutmann DH; Garbow JR. 2007. Detection and measurement of neurofibromatosis-1 mouse optic glioma in vivo. Neuroimage 35(4):1434-7. PubMed: 17383899MGI: J:122677
- Banerjee S; Byrd JN; Gianino SM; Harpstrite SE; Rodriguez FJ; Tuskan RG; Reilly KM; Piwnica-Worms DR; Gutmann DH. 2010. The Neurofibromatosis Type 1 Tumor Suppressor Controls Cell Growth by Regulating Signal Transducer and Activator of Transcription-3 Activity In vitro and In vivo. Cancer Res 70(4):1356-66. PubMed: 20124472MGI: J:157155
- Banerjee S; Crouse NR; Emnett RJ; Gianino SM; Gutmann DH. 2011. Neurofibromatosis-1 regulates mTOR-mediated astrocyte growth and glioma formation in a TSC/Rheb-independent manner. Proc Natl Acad Sci U S A 108(38):15996-6001. PubMed: 21896734MGI: J:176586
- Breunig JJ; Levy R; Antonuk CD; Molina J; Dutra-Clarke M; Park H; Akhtar AA; Kim GB; Hu X; Bannykh SI; Verhaak RG; Danielpour M. 2015. Ets Factors Regulate Neural Stem Cell Depletion and Gliogenesis in Ras Pathway Glioma. Cell Rep 12(2):258-71. PubMed: 26146073MGI: J:224846
- Brown JA; Diggs-Andrews KA; Gianino SM; Gutmann DH. 2012. Neurofibromatosis-1 heterozygosity impairs CNS neuronal morphology in a cAMP/PKA/ROCK-dependent manner. Mol Cell Neurosci 49(1):13-22. PubMed: 21903164MGI: J:189363
- Brown JA; Emnett RJ; White CR; Yuede CM; Conyers SB; O'Malley KL; Wozniak DF; Gutmann DH. 2010. Reduced striatal dopamine underlies the attention system dysfunction in neurofibromatosis-1 mutant mice. Hum Mol Genet 19(22):4515-28. PubMed: 20826448MGI: J:165138
- Brown JA; Gianino SM; Gutmann DH. 2010. Defective cAMP generation underlies the sensitivity of CNS neurons to neurofibromatosis-1 heterozygosity. J Neurosci 30(16):5579-89. PubMed: 20410111MGI: J:159840
- Brown JA; Xu J; Diggs-Andrews KA; Wozniak DF; Mach RH; Gutmann DH. 2011. PET imaging for attention deficit preclinical drug testing in neurofibromatosis-1 mice. Exp Neurol 232(2):333-8. PubMed: 21963652MGI: J:178468
- Chang T; Krisman K; Theobald EH; Xu J; Akutagawa J; Lauchle JO; Kogan S; Braun BS; Shannon K. 2013. Sustained MEK inhibition abrogates myeloproliferative disease in Nf1 mutant mice. J Clin Invest 123(1):335-9. PubMed: 23221337MGI: J:194161
- Chen J; Li Y; Yu TS; McKay RM; Burns DK; Kernie SG; Parada LF. 2012. A restricted cell population propagates glioblastoma growth after chemotherapy. Nature 488(7412):522-6. PubMed: 22854781MGI: J:186769
- Chen YH; Gianino SM; Gutmann DH. 2015. Neurofibromatosis-1 regulation of neural stem cell proliferation and multilineage differentiation operates through distinct RAS effector pathways. Genes Dev 29(16):1677-82. PubMed: 26272820MGI: J:226180
- Chen YH; Gutmann DH. 2014. The molecular and cell biology of pediatric low-grade gliomas. Oncogene 33(16):2019-26. PubMed: 23624918MGI: J:212366
- Chen Z; Liu C; Patel AJ; Liao CP; Wang Y; Le LQ. 2014. Cells of origin in the embryonic nerve roots for NF1-associated plexiform neurofibroma. Cancer Cell 26(5):695-706. PubMed: 25446898MGI: J:217465
- Cui Y; Costa RM; Murphy GG; Elgersma Y; Zhu Y; Gutmann DH; Parada LF; Mody I; Silva AJ. 2008. Neurofibromin regulation of ERK signaling modulates GABA release and learning. Cell 135(3):549-60. PubMed: 18984165MGI: J:147614
- Cutts BA; Sjogren AK; Andersson KM; Wahlstrom AM; Karlsson C; Swolin B; Bergo MO. 2009. Nf1 deficiency cooperates with oncogenic K-RAS to induce acute myeloid leukemia in mice. Blood 114(17):3629-32. PubMed: 19710506MGI: J:153839
- Dasgupta B; Dugan LL; Gutmann DH. 2003. The neurofibromatosis 1 gene product neurofibromin regulates pituitary adenylate cyclase-activating polypeptide-mediated signaling in astrocytes. J Neurosci 23(26):8949-54. PubMed: 14523097MGI: J:120166
- Dasgupta B; Li W; Perry A; Gutmann DH. 2005. Glioma formation in neurofibromatosis 1 reflects preferential activation of K-RAS in astrocytes. Cancer Res 65(1):236-45. PubMed: 15665300MGI: J:95509
- Dasgupta B; Yi Y; Chen DY; Weber JD; Gutmann DH. 2005. Proteomic analysis reveals hyperactivation of the mammalian target of rapamycin pathway in neurofibromatosis 1-associated human and mouse brain tumors. Cancer Res 65(7):2755-60. PubMed: 15805275MGI: J:97360
- Dasgupta B; Yi Y; Hegedus B; Weber JD; Gutmann DH. 2005. Cerebrospinal fluid proteomic analysis reveals dysregulation of methionine aminopeptidase-2 expression in human and mouse neurofibromatosis 1-associated glioma. Cancer Res 65(21):9843-50. PubMed: 16267007MGI: J:102692
- Deo M; Huang JL; Fuchs H; de Angelis MH; Van Raamsdonk CD. 2013. Differential effects of neurofibromin gene dosage on melanocyte development. J Invest Dermatol 133(1):49-58. PubMed: 22810304MGI: J:196495
- Dodd RD; Mito JK; Eward WC; Chitalia R; Sachdeva M; Ma Y; Barretina J; Dodd L; Kirsch DG. 2013. NF1 deletion generates multiple subtypes of soft-tissue sarcoma that respond to MEK inhibition. Mol Cancer Ther 12(9):1906-17. PubMed: 23858101MGI: J:234760
- El Khassawna T; Toben D; Kolanczyk M; Schmidt-Bleek K; Koennecke I; Schell H; Mundlos S; Duda GN. 2012. Deterioration of fracture healing in the mouse model of NF1 long bone dysplasia. Bone 51(4):651-60. PubMed: 22868293MGI: J:193350
- El-Hoss J; Sullivan K; Cheng T; Yu NY; Bobyn JD; Peacock L; Mikulec K; Baldock P; Alexander IE; Schindeler A; Little DG. 2012. A murine model of neurofibromatosis type 1 tibial pseudarthrosis featuring proliferative fibrous tissue and osteoclast-like cells. J Bone Miner Res 27(1):68-78. PubMed: 21956219MGI: J:226592
- Elefteriou F; Benson MD; Sowa H; Starbuck M; Liu X; Ron D; Parada LF; Karsenty G. 2006. ATF4 mediation of NF1 functions in osteoblast reveals a nutritional basis for congenital skeletal dysplasiae. Cell Metab 4(6):441-51. PubMed: 17141628MGI: J:129752
- Galvao RP; Kasina A; McNeill RS; Harbin JE; Foreman O; Verhaak RG; Nishiyama A; Miller CR; Zong H. 2014. Transformation of quiescent adult oligodendrocyte precursor cells into malignant glioma through a multistep reactivation process. Proc Natl Acad Sci U S A 111(40):E4214-23. PubMed: 25246577MGI: J:216462
- Gitler AD; Kong Y; Choi JK; Zhu Y; Pear WS; Epstein JA. 2004. Tie2-Cre-induced inactivation of a conditional mutant Nf1 allele in mouse results in a myeloproliferative disorder that models juvenile myelomonocytic leukemia. Pediatr Res 55(4):581-4. PubMed: 14739366MGI: J:88133
- Gitler AD; Zhu Y; Ismat FA; Lu MM; Yamauchi Y; Parada LF; Epstein JA. 2003. Nf1 has an essential role in endothelial cells. Nat Genet 33(1):75-9. PubMed: 12469121MGI: J:80323
- Gregorian C; Nakashima J; Dry SM; Nghiemphu PL; Smith KB; Ao Y; Dang J; Lawson G; Mellinghoff IK; Mischel PS; Phelps M; Parada LF; Liu X; Sofroniew MV; Eilber FC; Wu H. 2009. PTEN dosage is essential for neurofibroma development and malignant transformation. Proc Natl Acad Sci U S A 106(46):19479-84. PubMed: 19846776MGI: J:154673
- Hegedus B; Banerjee D; Yeh TH; Rothermich S; Perry A; Rubin JB; Garbow JR; Gutmann DH. 2008. Preclinical cancer therapy in a mouse model of neurofibromatosis-1 optic glioma. Cancer Res 68(5):1520-8. PubMed: 18316617MGI: J:132860
- Hegedus B; Dasgupta B; Shin JE; Emnett RJ; Hart-Mahon EK; Elghazi L; Bernal-Mizrachi E; Gutmann DH. 2007. Neurofibromatosis-1 regulates neuronal and glial cell differentiation from neuroglial progenitors in vivo by both cAMP- and Ras-dependent mechanisms. Cell Stem Cell 1(4):443-57. PubMed: 18371380MGI: J:139866
- Hegedus B; Yeh TH; Lee da Y; Emnett RJ; Li J; Gutmann DH. 2008. Neurofibromin regulates somatic growth through the hypothalamic-pituitary axis. Hum Mol Genet 17(19):2956-66. PubMed: 18614544MGI: J:138868
- Huse JT; Holland EC. 2009. Genetically engineered mouse models of brain cancer and the promise of preclinical testing. Brain Pathol 19(1):132-43. PubMed: 19076778MGI: J:173443
- Ismat FA; Xu J; Lu MM; Epstein JA. 2006. The neurofibromin GAP-related domain rescues endothelial but not neural crest development in Nf1 mice. J Clin Invest 116(9):2378-84. PubMed: 16906226MGI: J:114455
- Jessen WJ; Miller SJ; Jousma E; Wu J; Rizvi TA; Brundage ME; Eaves D; Widemann B; Kim MO; Dombi E; Sabo J; Hardiman Dudley A; Niwa-Kawakita M; Page GP; Giovannini M; Aronow BJ; Cripe TP; Ratner N. 2013. MEK inhibition exhibits efficacy in human and mouse neurofibromatosis tumors. J Clin Invest 123(1):340-7. PubMed: 23221341MGI: J:194157
- Joseph NM; Mosher JT; Buchstaller J; Snider P; McKeever PE; Lim M; Conway SJ; Parada LF; Zhu Y; Morrison SJ. 2008. The loss of Nf1 transiently promotes self-renewal but not tumorigenesis by neural crest stem cells. Cancer Cell 13(2):129-40. PubMed: 18242513MGI: J:131914
- Karolak MR; Yang X; Elefteriou F. 2015. FGFR1 signaling in hypertrophic chondrocytes is attenuated by the Ras-GAP neurofibromin during endochondral bone formation. Hum Mol Genet 24(9):2552-64. PubMed: 25616962MGI: J:220867
- Keng VW; Rahrmann EP; Watson AL; Tschida BR; Moertel CL; Jessen WJ; Rizvi TA; Collins MH; Ratner N; Largaespada DA. 2012. PTEN and NF1 inactivation in Schwann cells produces a severe phenotype in the peripheral nervous system that promotes the development and malignant progression of peripheral nerve sheath tumors. Cancer Res 72(13):3405-13. PubMed: 22700876MGI: J:189280
- Kim A; Morgan K; Hasz DE; Wiesner SM; Lauchle JO; Geurts JL; Diers MD; Le DT; Kogan SC; Parada LF; Shannon K; Largaespada DA. 2007. Beta common receptor inactivation attenuates myeloproliferative disease in Nf1 mutant mice. Blood 109(4):1687-91. PubMed: 17090653MGI: J:123850
- Kim E; Wang Y; Kim SJ; Bornhorst M; Jecrois ES; Anthony TE; Wang C; Li YE; Guan JL; Murphy GG; Zhu Y. 2014. Transient inhibition of the ERK pathway prevents cerebellar developmental defects and improves long-term motor functions in murine models of neurofibromatosis type 1. Elife 3:. PubMed: 25535838MGI: J:228845
- Kim KY; Ju WK; Hegedus B; Gutmann DH; Ellisman MH. 2010. Ultrastructural characterization of the optic pathway in a mouse model of neurofibromatosis-1 optic glioma. Neuroscience 170(1):178-88. PubMed: 20600672MGI: J:165209
- Kolanczyk M; Kossler N; Kuhnisch J; Lavitas L; Stricker S; Wilkening U; Manjubala I; Fratzl P; Sporle R; Herrmann BG; Parada LF; Kornak U; Mundlos S. 2007. Multiple roles for neurofibromin in skeletal development and growth. Hum Mol Genet 16(8):874-86. PubMed: 17317783MGI: J:121700
- Kossler N; Stricker S; Rodelsperger C; Robinson PN; Kim J; Dietrich C; Osswald M; Kuhnisch J; Stevenson DA; Braun T; Mundlos S; Kolanczyk M. 2011. Neurofibromin (Nf1) is required for skeletal muscle development. Hum Mol Genet 20(14):2697-709. PubMed: 21478499MGI: J:173779
- Kuhnisch J; Seto J; Lange C; Schrof S; Stumpp S; Kobus K; Grohmann J; Kossler N; Varga P; Osswald M; Emmerich D; Tinschert S; Thielemann F; Duda G; Seifert W; El Khassawna T; Stevenson DA; Elefteriou F; Kornak U; Raum K; Fratzl P; Mundlos S; Kolanczyk M. 2014. Multiscale, converging defects of macro-porosity, microstructure and matrix mineralization impact long bone fragility in NF1. PLoS One 9(1):e86115. PubMed: 24465906MGI: J:212331
- Kuhnisch J; Seto J; Lange C; Stumpp S; Kobus K; Grohmann J; Elefteriou F; Fratzl P; Mundlos S; Kolanczyk M. 2014. Neurofibromin inactivation impairs osteocyte development in Nf1Prx1 and Nf1Col1 mouse models. Bone 66:155-62. PubMed: 24947449MGI: J:215491
- Kwon CH; Zhao D; Chen J; Alcantara S; Li Y; Burns DK; Mason RP; Lee EY; Wu H; Parada LF. 2008. Pten haploinsufficiency accelerates formation of high-grade astrocytomas. Cancer Res 68(9):3286-94. PubMed: 18451155MGI: J:134611
- Lasater EA; Li F; Bessler WK; Estes ML; Vemula S; Hingtgen CM; Dinauer MC; Kapur R; Conway SJ; Ingram DA Jr. 2010. Genetic and cellular evidence of vascular inflammation in neurofibromin-deficient mice and humans. J Clin Invest 120(3):859-70. PubMed: 20160346MGI: J:158570
- Lauchle JO; Kim D; Le DT; Akagi K; Crone M; Krisman K; Warner K; Bonifas JM; Li Q; Coakley KM; Diaz-Flores E; Gorman M; Przybranowski S; Tran M; Kogan SC; Roose JP; Copeland NG; Jenkins NA; Parada L; Wolff L; Sebolt-Leopold J; Shannon K. 2009. Response and resistance to MEK inhibition in leukaemias initiated by hyperactive Ras. Nature 461(7262):411-4. PubMed: 19727076MGI: J:152381
- Le DT; Kong N; Zhu Y; Lauchle JO; Aiyigari A; Braun BS; Wang E; Kogan SC; Le Beau MM; Parada L; Shannon KM. 2004. Somatic inactivation of Nf1 in hematopoietic cells results in a progressive myeloproliferative disorder. Blood 103(11):4243-50. PubMed: 14982883MGI: J:90973
- Le LQ; Liu C; Shipman T; Chen Z; Suter U; Parada LF. 2011. Susceptible Stages in Schwann Cells for NF1-Associated Plexiform Neurofibroma Development. Cancer Res 71(13):4686-95. PubMed: 21551250MGI: J:173624
- Le LQ; Shipman T; Burns DK; Parada LF. 2009. Cell of origin and microenvironment contribution for NF1-associated dermal neurofibromas. Cell Stem Cell 4(5):453-63. PubMed: 19427294MGI: J:149832
- Lee da Y; Gianino SM; Gutmann DH. 2012. Innate neural stem cell heterogeneity determines the patterning of glioma formation in children. Cancer Cell 22(1):131-8. PubMed: 22789544MGI: J:191011
- Lee da Y; Yeh TH; Emnett RJ; White CR; Gutmann DH. 2010. Neurofibromatosis-1 regulates neuroglial progenitor proliferation and glial differentiation in a brain region-specific manner. Genes Dev 24(20):2317-29. PubMed: 20876733MGI: J:164880
- Li F; Downing BD; Smiley LC; Mund JA; Distasi MR; Bessler WK; Sarchet KN; Hinds DM; Kamendulis LM; Hingtgen CM; Case J; Clapp DW; Conway SJ; Stansfield BK; Ingram DA Jr. 2014. Neurofibromin-deficient myeloid cells are critical mediators of aneurysm formation in vivo. Circulation 129(11):1213-24. PubMed: 24370551MGI: J:219312
- Li Y; Li Y; McKay RM; Riethmacher D; Parada LF. 2012. Neurofibromin modulates adult hippocampal neurogenesis and behavioral effects of antidepressants. J Neurosci 32(10):3529-39. PubMed: 22399775MGI: J:182728
- Li YJ; Takizawa H; Azuma A; Kohyama T; Yamauchi Y; Takahashi S; Yamamoto M; Kawada T; Kudoh S; Sugawara I. 2008. Disruption of Nrf2 enhances susceptibility to airway inflammatory responses induced by low-dose diesel exhaust particles in mice. Clin Immunol 128(3):366-73. PubMed: 18614404MGI: J:138869
- Lin L; Chen J; Richardson JA; Parada LF. 2009. Mice lacking neurofibromin develop gastric hyperplasia. Am J Physiol Gastrointest Liver Physiol 297(4):G751-61. PubMed: 19661150MGI: J:209264
- Lush ME; Li Y; Kwon CH; Chen J; Parada LF. 2008. Neurofibromin is required for barrel formation in the mouse somatosensory cortex. J Neurosci 28(7):1580-7. PubMed: 18272679MGI: J:132220
- Maertens O; Johnson B; Hollstein P; Frederick DT; Cooper ZA; Messiaen L; Bronson RT; McMahon M; Granter S; Flaherty K; Wargo JA; Marais R; Cichowski K. 2013. Elucidating distinct roles for NF1 in melanomagenesis. Cancer Discov 3(3):338-49. PubMed: 23171796MGI: J:198252
- Mayes DA; Rizvi TA; Cancelas JA; Kolasinski NT; Ciraolo GM; Stemmer-Rachamimov AO; Ratner N. 2011. Perinatal or Adult Nf1 Inactivation Using Tamoxifen-Inducible PlpCre Each Cause Neurofibroma Formation. Cancer Res 71(13):4675-85. PubMed: 21551249MGI: J:173625
- Mo W; Chen J; Patel A; Zhang L; Chau V; Li Y; Cho W; Lim K; Xu J; Lazar AJ; Creighton CJ; Bolshakov S; McKay RM; Lev D; Le LQ; Parada LF. 2013. CXCR4/CXCL12 Mediate Autocrine Cell- Cycle Progression in NF1-Associated Malignant Peripheral Nerve Sheath Tumors. Cell 152(5):1077-90. PubMed: 23434321MGI: J:193957
- Oliver JA; Lapinski PE; Lubeck BA; Turner JS; Parada LF; Zhu Y; King PD. 2013. The Ras GTPase-activating protein neurofibromin 1 promotes the positive selection of thymocytes. Mol Immunol 55(3-4):292-302. PubMed: 23522726MGI: J:202145
- Ono K; Karolak MR; Ndong Jde L; Wang W; Yang X; Elefteriou F. 2013. The Ras-GTPase activity of neurofibromin restrains ERK-dependent FGFR signaling during endochondral bone formation. Hum Mol Genet 22(15):3048-62. PubMed: 23571107MGI: J:198529
- Parrinello S; Noon LA; Harrisingh MC; Digby PW; Rosenberg LH; Cremona CA; Echave P; Flanagan AM; Parada LF; Lloyd AC. 2008. NF1 loss disrupts Schwann cell-axonal interactions: a novel role for semaphorin 4F. Genes Dev 22(23):3335-48. PubMed: 19056885MGI: J:142040
- Patmore DM; Welch S; Fulkerson PC; Wu J; Choi K; Eaves D; Kordich JJ; Collins MH; Cripe TP; Ratner N. 2012. In vivo regulation of TGF-beta by R-Ras2 revealed through loss of the RasGAP protein NF1. Cancer Res 72(20):5317-27. PubMed: 22918885MGI: J:191804
- Pelle DW; Peacock JD; Schmidt CL; Kampfschulte K; Scholten DJ 2nd; Russo SS; Easton KJ; Steensma MR. 2014. Genetic and functional studies of the intervertebral disc: a novel murine intervertebral disc model. PLoS One 9(12):e112454. PubMed: 25474689MGI: J:225682
- Prada CE; Jousma E; Rizvi TA; Wu J; Dunn RS; Mayes DA; Cancelas JA; Dombi E; Kim MO; West BL; Bollag G; Ratner N. 2013. Neurofibroma-associated macrophages play roles in tumor growth and response to pharmacological inhibition. Acta Neuropathol 125(1):159-68. PubMed: 23099891MGI: J:211760
- Price RL; Song J; Bingmer K; Kim TH; Yi JY; Nowicki MO; Mo X; Hollon T; Murnan E; Alvarez-Breckenridge C; Fernandez S; Kaur B; Rivera A; Oglesbee M; Cook C; Chiocca EA; Kwon CH. 2013. Cytomegalovirus contributes to glioblastoma in the context of tumor suppressor mutations. Cancer Res 73(11):3441-50. PubMed: 23729642MGI: J:199096
- Rhodes SD; Wu X; He Y; Chen S; Yang H; Staser KW; Wang J; Zhang P; Jiang C; Yokota H; Dong R; Peng X; Yang X; Murthy S; Azhar M; Mohammad KS; Xu M; Guise TA; Yang FC. 2013. Hyperactive transforming growth factor-beta1 signaling potentiates skeletal defects in a neurofibromatosis type 1 mouse model. J Bone Miner Res 28(12):2476-89. PubMed: 23703870MGI: J:231256
- Rhodes SD; Zhang W; Yang D; Yang H; Chen S; Wu X; Li X; Yang X; Mohammad KS; Guise TA; Bergner AL; Stevenson DA; Yang FC. 2015. Dystrophic spinal deformities in a neurofibromatosis type 1 murine model. PLoS One 10(3):e0119093. PubMed: 25786243MGI: J:229201
- Ribeiro S; Napoli I; White IJ; Parrinello S; Flanagan AM; Suter U; Parada LF; Lloyd AC. 2013. Injury signals cooperate with Nf1 loss to relieve the tumor-suppressive environment of adult peripheral nerve. Cell Rep 5(1):126-36. PubMed: 24075988MGI: J:203786
- Romero MI; Lin L; Lush ME; Lei L; Parada LF; Zhu Y. 2007. Deletion of Nf1 in neurons induces increased axon collateral branching after dorsal root injury. J Neurosci 27(8):2124-34. PubMed: 17314307MGI: J:118374
- Sachdeva M; Dodd RD; Huang Z; Grenier C; Ma Y; Lev DC; Cardona DM; Murphy SK; Kirsch DG. 2015. Epigenetic silencing of Kruppel like factor-3 increases expression of pro-metastatic miR-182. Cancer Lett 369(1):202-11. PubMed: 26314219MGI: J:225962
- Sanchez-Ortiz E; Cho W; Nazarenko I; Mo W; Chen J; Parada LF. 2014. NF1 regulation of RAS/ERK signaling is required for appropriate granule neuron progenitor expansion and migration in cerebellar development. Genes Dev 28(21):2407-20. PubMed: 25367036MGI: J:217419
- Sharma R; Wu X; Rhodes SD; Chen S; He Y; Yuan J; Li J; Yang X; Li X; Jiang L; Kim ET; Stevenson DA; Viskochil D; Xu M; Yang FC. 2013. Hyperactive Ras/MAPK signaling is critical for tibial nonunion fracture in neurofibromin-deficient mice. Hum Mol Genet 22(23):4818-28. PubMed: 23863460MGI: J:202235
- Shchors K; Massaras A; Hanahan D. 2015. Dual Targeting of the Autophagic Regulatory Circuitry in Gliomas with Repurposed Drugs Elicits Cell-Lethal Autophagy and Therapeutic Benefit. Cancer Cell 28(4):456-71. PubMed: 26412325MGI: J:225946
- Shilyansky C; Karlsgodt KH; Cummings DM; Sidiropoulou K; Hardt M; James AS; Ehninger D; Bearden CE; Poirazi P; Jentsch JD; Cannon TD; Levine MS; Silva AJ. 2010. Neurofibromin regulates corticostriatal inhibitory networks during working memory performance. Proc Natl Acad Sci U S A 107(29):13141-6. PubMed: 20624961MGI: J:162376
- Smithson LJ; Gutmann DH. 2016. Proteomic analysis reveals GIT1 as a novel mTOR complex component critical for mediating astrocyte survival. Genes Dev 30(12):1383-8. PubMed: 27340174MGI: J:233300
- Solga AC; Gianino SM; Gutmann DH. 2014. NG2-cells are not the cell of origin for murine neurofibromatosis-1 (Nf1) optic glioma. Oncogene 33(3):289-99. PubMed: 23318450MGI: J:204871
- Stansfield BK; Bessler WK; Mali R; Mund JA; Downing B; Li F; Sarchet KN; Distasi MR; Conway SJ; Kapur R; Ingram DA Jr. 2013. Heterozygous inactivation of the Nf1 gene in myeloid cells enhances neointima formation via a rosuvastatin-sensitive cellular pathway. Hum Mol Genet 22(5):977-88. PubMed: 23197650MGI: J:192569
- Staser K; Park SJ; Rhodes SD; Zeng Y; He YZ; Shew MA; Gehlhausen JR; Cerabona D; Menon K; Chen S; Sun Z; Yuan J; Ingram DA; Nalepa G; Yang FC; Clapp DW. 2013. Normal hematopoiesis and neurofibromin-deficient myeloproliferative disease require Erk. J Clin Invest 123(1):329-34. PubMed: 23221339MGI: J:194159
- Su W; Xing R; Guha A; Gutmann DH; Sherman LS. 2007. Mice with GFAP-targeted loss of neurofibromin demonstrate increased axonal MET expression with aging. Glia 55(7):723-33. PubMed: 17348023MGI: J:156097
- Sullivan K; El-Hoss J; Quinlan KG; Deo N; Garton F; Seto JT; Gdalevitch M; Turner N; Cooney GJ; Kolanczyk M; North KN; Little DG; Schindeler A. 2014. NF1 is a critical regulator of muscle development and metabolism. Hum Mol Genet 23(5):1250-9. PubMed: 24163128MGI: J:206219
- Sun T; Warrington NM; Luo J; Brooks MD; Dahiya S; Snyder SC; Sengupta R; Rubin JB. 2014. Sexually dimorphic RB inactivation underlies mesenchymal glioblastoma prevalence in males. J Clin Invest 124(9):4123-33. PubMed: 25083989MGI: J:215767
- Switon K; Kotulska K; Janusz-Kaminska A; Zmorzynska J; Jaworski J. 2017. Molecular neurobiology of mTOR. Neuroscience 341:112-153. PubMed: 27889578MGI: J:238268
- Tan M; Zhao Y; Kim SJ; Liu M; Jia L; Saunders TL; Zhu Y; Sun Y. 2011. SAG/RBX2/ROC2 E3 Ubiquitin Ligase Is Essential for Vascular and Neural Development by Targeting NF1 for Degradation. Dev Cell 21(6):1062-76. PubMed: 22118770MGI: J:178926
- Toonen JA; Solga AC; Ma Y; Gutmann DH. 2017. Estrogen activation of microglia underlies the sexually dimorphic differences in Nf1 optic glioma-induced retinal pathology. J Exp Med 214(1):17-25. PubMed: 27923908MGI: J:238781
- Wang W; Nyman JS; Moss HE; Gutierrez G; Mundy GR; Yang X; Elefteriou F. 2010. Local low-dose lovastatin delivery improves the bone-healing defect caused by Nf1 loss of function in osteoblasts. J Bone Miner Res 25(7):1658-67. PubMed: 20200958MGI: J:183363
- Wang W; Nyman JS; Ono K; Stevenson DA; Yang X; Elefteriou F. 2011. Mice lacking Nf1 in osteochondroprogenitor cells display skeletal dysplasia similar to patients with neurofibromatosis type I. Hum Mol Genet 20(20):3910-24. PubMed: 21757497MGI: J:175684
- Warrington NM; Gianino SM; Jackson E; Goldhoff P; Garbow JR; Piwnica-Worms D; Gutmann DH; Rubin JB. 2010. Cyclic AMP suppression is sufficient to induce gliomagenesis in a mouse model of neurofibromatosis-1. Cancer Res 70(14):5717-27. PubMed: 20551058MGI: J:162482
- Warrington NM; Sun T; Luo J; McKinstry RC; Parkin PC; Ganzhorn S; Spoljaric D; Albers AC; Merkelson A; Stewart DR; Stevenson DA; Viskochil D; Druley TE; Forys JT; Reilly KM; Fisher MJ; Tabori U; Allen JC; Schiffman JD; Gutmann DH; Rubin JB. 2015. The cyclic AMP pathway is a sex-specific modifier of glioma risk in type I neurofibromatosis patients. Cancer Res 75(1):16-21. PubMed: 25381154MGI: J:218215
- Warrington NM; Woerner BM; Daginakatte GC; Dasgupta B; Perry A; Gutmann DH; Rubin JB. 2007. Spatiotemporal Differences in CXCL12 Expression and Cyclic AMP Underlie the Unique Pattern of Optic Glioma Growth in Neurofibromatosis Type 1. Cancer Res 67(18):8588-95. PubMed: 17875698MGI: J:124875
- Wong JC; Zhang Y; Lieuw KH; Tran MT; Forgo E; Weinfurtner K; Alzamora P; Kogan SC; Akagi K; Wolff L; Le Beau MM; Killeen N; Shannon K. 2010. Use of chromosome engineering to model a segmental deletion of chromosome band 7q22 found in myeloid malignancies. Blood 115(22):4524-32. PubMed: 20233966MGI: J:161558
- Wozniak DF; Diggs-Andrews KA; Conyers S; Yuede CM; Dearborn JT; Brown JA; Tokuda K; Izumi Y; Zorumski CF; Gutmann DH. 2013. Motivational disturbances and effects of L-dopa administration in neurofibromatosis-1 model mice. PLoS One 8(6):e66024. PubMed: 23762458MGI: J:203309
- Wu J; Dombi E; Jousma E; Scott Dunn R; Lindquist D; Schnell BM; Kim MO; Kim A; Widemann BC; Cripe TP; Ratner N. 2012. Preclincial testing of sorafenib and RAD001 in the Nf(flox/flox) ;DhhCre mouse model of plexiform neurofibroma using magnetic resonance imaging. Pediatr Blood Cancer 58(2):173-80. PubMed: 21319287MGI: J:215873
- Wu J; Patmore DM; Jousma E; Eaves DW; Breving K; Patel AV; Schwartz EB; Fuchs JR; Cripe TP; Stemmer-Rachamimov AO; Ratner N. 2014. EGFR-STAT3 signaling promotes formation of malignant peripheral nerve sheath tumors. Oncogene 33(2):173-80. PubMed: 23318430MGI: J:204875
- Wu J; Williams JP; Rizvi TA; Kordich JJ; Witte D; Meijer D; Stemmer-Rachamimov AO; Cancelas JA; Ratner N. 2008. Plexiform and dermal neurofibromas and pigmentation are caused by Nf1 loss in desert hedgehog-expressing cells. Cancer Cell 13(2):105-16. PubMed: 18242511MGI: J:131916
- Xu J; Ismat FA; Wang T; Lu MM; Antonucci N; Epstein JA. 2009. Cardiomyocyte-specific loss of neurofibromin promotes cardiac hypertrophy and dysfunction. Circ Res 105(3):304-11. PubMed: 19574548MGI: J:164937
- Xu J; Ismat FA; Wang T; Yang J; Epstein JA. 2007. NF1 regulates a Ras-dependent vascular smooth muscle proliferative injury response. Circulation 116(19):2148-56. PubMed: 17967772MGI: J:142994
- Yeh TH; Lee da Y; Gianino SM; Gutmann DH. 2009. Microarray analyses reveal regional astrocyte heterogeneity with implications for neurofibromatosis type 1 (NF1)-regulated glial proliferation. Glia 57(11):1239-49. PubMed: 19191334MGI: J:156205
- Yin B; Delwel R; Valk PJ; Wallace MR; Loh ML; Shannon KM; Largaespada DA. 2009. A retroviral mutagenesis screen reveals strong cooperation between Bcl11a overexpression and loss of the Nf1 tumor suppressor gene. Blood 113(5):1075-85. PubMed: 18948576MGI: J:144622
- Zhang W; Rhodes SD; Zhao L; He Y; Zhang Y; Shen Y; Yang D; Wu X; Li X; Yang X; Park SJ; Chen S; Turner C; Yang FC. 2011. Primary osteopathy of vertebrae in a neurofibromatosis type 1 murine model. Bone 48(6):1378-87. PubMed: 21439418MGI: J:172512
- Zheng H; Chang L; Patel N; Yang J; Lowe L; Burns DK; Zhu Y. 2008. Induction of abnormal proliferation by nonmyelinating schwann cells triggers neurofibroma formation. Cancer Cell 13(2):117-28. PubMed: 18242512MGI: J:131915
- Zhu Y; Ghosh P; Charnay P; Burns DK; Parada LF. 2002. Neurofibromas in NF1: Schwann cell origin and role of tumor environment. Science 296(5569):920-2. PubMed: 11988578MGI: J:76359
- Zhu Y; Guignard F; Zhao D; Liu L; Burns DK; Mason RP; Messing A; Parada LF. 2005. Early inactivation of p53 tumor suppressor gene cooperating with NF1 loss induces malignant astrocytoma. Cancer Cell 8(2):119-30. PubMed: 16098465MGI: J:101868
- Zhu Y; Harada T; Liu L; Lush ME; Guignard F; Harada C; Burns DK; Bajenaru ML; Gutmann DH; Parada LF. 2005. Inactivation of NF1 in CNS causes increased glial progenitor proliferation and optic glioma formation. Development 132(24):5577-88. PubMed: 16314489MGI: J:104342
- de la Croix Ndong J; Makowski AJ; Uppuganti S; Vignaux G; Ono K; Perrien DS; Joubert S; Baglio SR; Granchi D; Stevenson DA; Rios JJ; Nyman JS; Elefteriou F. 2014. Asfotase-alpha improves bone growth, mineralization and strength in mouse models of neurofibromatosis type-1. Nat Med 20(8):904-10. PubMed: 24997609MGI: J:227603