JAX Mouse Strain Datasheet - 006999

STOCK Dbt^tm1Geh Tg(Cebpb-tTA)5Bjd Tg(tetO-DBT)A1Geh/J

Stock No:: 006999 |; MSUD murine model

Cryo Recovery

Overview

Also Known As: MSUD murine model

In these triple mutant mice the absence of keto acid dehydrogenase (BCKDH) activity and E2 protein in liver tissue due to the Dbt^tm1Geh knock-out mutation is rescued by the two transgenes, rendering this strain as a useful model for Maple Syrup Urine Disease (MSUD).

Donating Investigator

Dr. Gregg Homanics, University of Pittsburgh

Genetic overview

Genetic Background	Generation

Dbt^tm1Geh

Allele Type	Gene Symbol	Gene Name
Targeted (Null/Knockout)	Dbt	dihydrolipoamide branched chain transacylase E2

Tg(Cebpb-tTA)5Bjd

Allele Type
Transgenic (Transactivator)

Tg(tetO-DBT)A1Geh

Allele Type
Transgenic (Humanized sequence, Hypomorph, Inducible, Inserted expressed sequence)

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Research Applications

Metabolism Research
Research Tools

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Base Price

Starting at:

$2,595.00 Domestic price Cryo Recovery

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Details

Detailed Description

Mice homozygous for the Dbt (E2) targeted mutation and carrying both the LAP-tTA and TRE-E2 transgenes are viable and fertile. The E2-targeted mutation leads to absence of branched-chain keto acid dehydrogenase (BCKDH) activity and E2 protein in liver tissue, but this absence is rescued by the two transgenes: liver-directed expression of the modified human BCKDH E2 subunit from the complimentary "Tet-off" transgenes abrogates the severity of Maple Syrup Urine Disease (MSUD) phenotype observed in E2-deficient single mutant mice. Triple mutant mice are a model for intermediate MSUD (iMSUD); BCKDH activity is only 5-6% of that found in wildtype mice. This low level of BCKDH activity is sufficient to allow survival, but insufficient to normalize circulating branched chain amino acids levels. Because these mice have near normal amounts of E2 protein, but only 5-6% of normal BCKDH enzyme activity, it is probable that the c-myc tag at the carboxy-terminus of the human E2 transgene interferes with enzymatic activity. The donating investigator reports that addition of the tetracycline analog doxycycline does not affect MSUD phenotype rescue. These mutant mice may be useful in studying metabolic disease, biochemistry, and both the complete/classic and intermediate forms of Maple Syrup Urine Disease.

Development

The mutant allele of branched-chain keto acid dehydrogenase E2 subunit (Dbt gene) was created by Dr. Gregg Homanics (University of Pittsburgh) using a targeting vector designed to replace part of exon 4 and all of exon 5 with a PGKneo cassette. The construct was electroporated into (129X1/SvJ x 129S1/Sv)F1-derived R1 embryonic stem (ES) cells. Correctly targeted ES cells were microinjected into C57BL/6J blastocysts. Chimeric mice were bred to C57BL/6J to generate heterozygous mice. Heterozygotes were then bred to LAP-tTA transgenic mice on a mixed (NMRI outbred;FVB;C57BL/6J) background. These mice were created by Dr. Hermann Bujard (Universitat Heidelberg) to express tetracycline-controlled transactivator (tTA) under control of the rat liver-enriched activator protein promoter. The TRE-E2 (or tetO-DBT*) transgene, also created by Dr. Gregg Homanics, contains a tetracycline response element and minimal human CMV promoter controlling a human E2 subunit (DBT gene) cDNA which has been modified to contain a 4x alanine linker followed by a c-myc epitope tag at the carboxy terminus. After injection of the transgene into C57BL/6J embryos, founder line A mice were bred with E2-deficient, LAP-tTA double mutant mice to generate this triple mutant strain.

Expression Data

Expressed Gene	tTA, tetracycline-controlled transactivator, E. coli
Site of Expression	Expresses tTA at high levels in the liver; see Stock No. 003563
Expressed Gene	DBT, dihydrolipoamide branched chain transacylase E2, human
Expressed Gene	tTA, tetracycline-controlled transactivator, E. coli
Site of Expression	Expresses tTA at high levels in the liver; see Stock No. 003563

Control Suggestions

Wild-type from the colony

Additional Information

Considerations for Choosing Controls

Selected References

Homanics GE; Skvorak K; Ferguson C; Watkins S; Paul HS. 2006. Production and characterization of murine models of classic and intermediate maple syrup urine disease. BMC Med Genet 7:33. PubMed: 16579849 MGI: J:119973

View All References

Genetics

Dbt^tm1Geh

Allele Symbol: Dbt^tm1Geh

Allele Name	targeted mutation 1, Gregg E Homanics
Allele Type	Targeted (Null/Knockout)
Allele Synonym(s)	E2 KO
Gene Symbol and Name	Dbt, dihydrolipoamide branched chain transacylase E2
Gene Synonym(s)	BCATE2; BCKAD E2; BCKAD-E2; BCKADE2; BCOADC-E2; D3Wsu60e; D3Wsu60e; DNA segment, Chr 3, Wayne State University 60, expressed; E2; E2B; dihydrolipoyl transacylase; dihydrolipoyllysine-residue (2-methylpropanoyl)transferase
Site of Expression	The targeted mutation leads to absence of branched-chain keto acid dehydrogenase activity and E2 protein in liver tissue.
Strain of Origin	(129X1/SvJ x 129S1/Sv)F1-Kitl<+>
Chromosome	3
Molecular Note	A targeting vector was used to replace part of exon 4 and all of exon 5 with a PGKneo cassette.Southern blot and immunohistochemical analyses verify the absence of wild-type E2 transcripts and lack of E2 protein detection in homozygotes.

Tg(Cebpb-tTA)5Bjd

Allele Symbol: Tg(Cebpb-tTA)5Bjd

Allele Name	transgene insertion 5, Hermann Bujard
Allele Type	Transgenic (Transactivator)
Allele Synonym(s)	LAP-tTA; Tg(tTALap)5Uh; g(tTALap)5Bjd
Gene Symbol and Name	Tg(Cebpb-tTA)5Bjd, transgene insertion 5, Hermann Bujard
Gene Synonym(s)	LAP-tTA; Tg(tTALap)5Bjd; Tg(tTALap)5Bjd; Tg(tTALap)5Uh
Promoter	Cebpb, CCAAT/enhancer binding protein beta, rat
Expressed Gene	tTA, tetracycline-controlled transactivator, E. coli
Site of Expression	Expresses tTA at high levels in the liver; see Stock No. 003563
Strain of Origin	NMRI
Chromosome	UN
General Note	Transgenic mice on a C57BL/6J background are viable and fertile, and express tTA in the liver.
Molecular Note	This transgene contains the gene encoding the tetracycline regulated transactivator protein (tTA) driven by the rat Cebpb promoter (previously called liver-enriched activator protein).
Mutations Made By	Dr. Hermann Bujard, Universitt Heidelberg

Tg(tetO-DBT)A1Geh

Allele Symbol: Tg(tetO-DBT)A1Geh

Allele Name	transgene insertion A1, Gregg E Homanics
Allele Type	Transgenic (Humanized sequence, Hypomorph, Inducible, Inserted expressed sequence)
Allele Synonym(s)	TRE-E2 (line A)
Gene Symbol and Name	Tg(tetO-DBT)A1Geh, transgene insertion A1, Gregg E Homanics
Gene Synonym(s)	TRE-E2 (line A)
Promoter	tetO, tet operator,
Expressed Gene	DBT, dihydrolipoamide branched chain transacylase E2, human
Expressed Gene	tTA, tetracycline-controlled transactivator, E. coli
Site of Expression	Expresses tTA at high levels in the liver; see Stock No. 003563
Strain of Origin	C57BL/6J
Chromosome	UN
Molecular Note	The TRE-E2 (or tetO-DBT) transgene contains a tetracycline response element and minimal human CMV promoter controlling a human E2 subunit (DBT gene) cDNA which has been modified to contain a 4x alanine linker followed by a c-myc epitope tag at the carboxy terminus to facilitate detection. Levels of the human E2 protein are approximately equal to mouse E2 protein in livers of control mice. However, when crossed to animals that express a transgene encoding the tetracycline regulated transactivator protein (Tg(tTALap)5Bjd) and are homozygous for a knockout allele of mouse E2 (Dbt^tm1Geh) are treated with doxycycline, the E2 protein levels correlate with only ~5-6% of normal branched-chain keto acid dehydrogenase (BCKDH) activity. It is one hypothesis that addition of the c-myc tag interferes with BCKDH subunit assembly (ie. interaction the human E2 subunit with mouse E1 and E3 complexes), thus reducing enzymatic activity.

Disease/Phenotype

Disease Terms

Research Areas By Genotype

This mouse can be used to support research in many areas including:

Metabolism Research
- Enzyme Deficiency
Research Tools
- Cardiovascular Research
  - Tetop Tet System
- Metabolism Research
- Tet Expression Systems
  - tTA/rtTA Expressing Strains
  - tTA/rtTA Responsive Strains

Mammalian Phenotype Terms by Genotype

Genotype: Dbt^tm1Geh/Dbt^tm1Geh Tg(tetO-DBT)A1Geh/0 Tg(Cebpb-tTA)5Bjd/0
involves: 129S1/Sv * 129X1/SvJ * C57BL/6J * FVB * NMRI

mortality/aging

postnatal lethality
- approximately 4% of pups surviving to weaning have this genotype, compared to a theoretical survival rate or 14%, indicating partial rescue of the Dbt^tm1Geh phenotype

premature death
- transgenic mice survival is only ~16 weeks, when mice become moribund and are sacrificed

homeostasis/metabolism phenotype

abnormal circulating amino acid level
- BCAA/alanine (BCAA -branched-chain amino acids - leucine + isoleucine + valine) values are intermediate between controls and Dbt-null mice between 4-7 weeks of age
- blood levels of alanine, glutamate, and glutamine are reduced compared to controls, but greater than in Dbt^tm1Geh homozygous mice

abnormal enzyme/coenzyme activity
- levels of branched-chain keto acid dehydrogenase (BCKDH) activity are only 5-6% of controls levels, despite nearly equal protein levels, indicating suboptimal BCKDH activity of protein assembled with human E2

References

Homanics GE; Skvorak K; Ferguson C; Watkins S; Paul HS. 2006. Production and characterization of murine models of classic and intermediate maple syrup urine disease. BMC Med Genet 7:33. PubMed: 16579849 MGI: J:119973

Additional - Dbt^tm1Geh related

Skvorak KJ; Hager EJ; Arning E; Bottiglieri T; Paul HS; Strom SC; Homanics GE; Sun Q; Jansen EE; Jakobs C; Zinnanti WJ; Gibson KM. 2009. Hepatocyte transplantation (HTx) corrects selected neurometabolic abnormalities in murine intermediate maple syrup urine disease (iMSUD). Biochim Biophys Acta 1792(10):1004-10. PubMed: 19699299 MGI: J:164850

Additional - Tg(Cebpb-tTA)5Bjd related

Beer S; Komatsubara K; Bellovin DI; Kurobe M; Sylvester K; Felsher DW. 2008. Hepatotoxin-induced changes in the adult murine liver promote MYC-induced tumorigenesis. PLoS One 3(6):e2493. PubMed: 18560566 MGI: J:139884
Beer S; Zetterberg A; Ihrie RA; McTaggart RA; Yang Q; Bradon N; Arvanitis C; Attardi LD; Feng S; Ruebner B; Cardiff RD; Felsher DW. 2004. Developmental Context Determines Latency of MYC-Induced Tumorigenesis. PLoS Biol 2(11):E332. PubMed: 15455033 MGI: J:93372
Cairo S; Armengol C; De Reynies A; Wei Y; Thomas E; Renard CA; Goga A; Balakrishnan A; Semeraro M; Gresh L; Pontoglio M; Strick-Marchand H; Levillayer F; Nouet Y; Rickman D; Gauthier F; Branchereau S; Brugieres L; Laithier V; Bouvier R; Boman F; Basso G;Michiels JF; Hofman P; Arbez-Gindre F; Jouan H; Rousselet-Chapeau MC; Berrebi D; Marcellin L; Plenat F; Zachar D; Joubert M; Selves J; Pasquier D; Bioulac-Sage P; Grotzer M; Childs M; Fabre M; Buendia MA. 2008. Hepatic stem-like phenotype and interplay of Wnt/beta-catenin and Myc signaling in aggressive childhood liver cancer. Cancer Cell 14(6):471-84. PubMed: 19061838 MGI: J:142029
Cao Z; Fan-Minogue H; Bellovin DI; Yevtodiyenko A; Arzeno J; Yang Q; Gambhir SS; Felsher DW. 2011. MYC Phosphorylation, Activation, and Tumorigenic Potential in Hepatocellular Carcinoma Are Regulated by HMG-CoA Reductase. Cancer Res 71(6):2286-97. PubMed: 21262914 MGI: J:170758
Carpenter B; Lin Y; Stoll S; Raffai RL; McCuskey R; Wang R. 2005. VEGF is crucial for the hepatic vascular development required for lipoprotein uptake. Development 132(14):3293-303. PubMed: 15944181 MGI: J:100427
Chai X; Guo Y; Jiang M; Hu B; Li Z; Fan J; Deng M; Billiar TR; Kucera HR; Gaikwad NW; Xu M; Lu P; Yan J; Fu H; Liu Y; Yu L; Huang M; Zeng S; Xie W. 2015. Oestrogen sulfotransferase ablation sensitizes mice to sepsis. Nat Commun 6:7979. PubMed: 26259151 MGI: J:224997
Chow EK; Zhang XQ; Chen M; Lam R; Robinson E; Huang H; Schaffer D; Osawa E; Goga A; Ho D. 2011. Nanodiamond therapeutic delivery agents mediate enhanced chemoresistant tumor treatment. Sci Transl Med 3(73):73ra21. PubMed: 21389265 MGI: J:170992
Dickins RA; McJunkin K; Hernando E; Premsrirut PK; Krizhanovsky V; Burgess DJ; Kim SY; Cordon-Cardo C; Zender L; Hannon GJ; Lowe SW. 2007. Tissue-specific and reversible RNA interference in transgenic mice. Nat Genet 39(7):914-21. PubMed: 17572676 MGI: J:123006
Goga A; Yang D; Tward AD; Morgan DO; Bishop JM. 2007. Inhibition of CDK1 as a potential therapy for tumors over-expressing MYC. Nat Med 13(7):820-7. PubMed: 17589519 MGI: J:125802
Hasan MT; Schonig K; Berger S; Graewe W; Bujard H. 2001. Long-term, noninvasive imaging of regulated gene expression in living mice. Genesis 29(3):116-22. PubMed: 11252052 MGI: J:127660
Hasenfuss SC; Bakiri L; Thomsen MK; Hamacher R; Wagner EF. 2014. Activator Protein 1 transcription factor Fos-related antigen 1 (Fra-1) is dispensable for murine liver fibrosis, but modulates xenobiotic metabolism. Hepatology 59(1):261-73. PubMed: 23703832 MGI: J:213764
Hasenfuss SC; Bakiri L; Thomsen MK; Williams EG; Auwerx J; Wagner EF. 2014. Regulation of steatohepatitis and PPARgamma signaling by distinct AP-1 dimers. Cell Metab 19(1):84-95. PubMed: 24411941 MGI: J:210545
Hsu SH; Wang B; Kota J; Yu J; Costinean S; Kutay H; Yu L; Bai S; La Perle K; Chivukula RR; Mao H; Wei M; Clark KR; Mendell JR; Caligiuri MA; Jacob ST; Mendell JT; Ghoshal K. 2012. Essential metabolic, anti-inflammatory, and anti-tumorigenic functions of miR-122 in liver. J Clin Invest 122(8):2871-83. PubMed: 22820288 MGI: J:190067
Hu S; Balakrishnan A; Bok RA; Anderton B; Larson PE; Nelson SJ; Kurhanewicz J; Vigneron DB; Goga A. 2011. 13C-pyruvate imaging reveals alterations in glycolysis that precede c-Myc-induced tumor formation and regression. Cell Metab 14(1):131-42. PubMed: 21723511 MGI: J:176076
Ivanovska I; Zhang C; Liu AM; Wong KF; Lee NP; Lewis P; Philippar U; Bansal D; Buser C; Scott M; Mao M; Poon RT; Fan ST; Cleary MA; Luk JM; Dai H. 2011. Gene signatures derived from a c-MET-driven liver cancer mouse model predict survival of patients with hepatocellular carcinoma. PLoS One 6(9):e24582. PubMed: 21949730 MGI: J:177679
Kim A; Joseph S; Khan A; Epstein CJ; Sobel R; Huang TT. 2010. Enhanced expression of mitochondrial superoxide dismutase leads to prolonged in vivo cell cycle progression and up-regulation of mitochondrial thioredoxin. Free Radic Biol Med 48(11):1501-12. PubMed: 20188820 MGI: J:160351
Kistner A; Gossen M; Zimmermann F; Jerecic J; Ullmer C; Lubbert H; Bujard H. 1996. Doxycycline-mediated quantitative and tissue-specific control of gene expression in transgenic mice. Proc Natl Acad Sci U S A 93(20):10933-8. PubMed: 8855286 MGI: J:93000
Kornmann B; Schaad O; Bujard H; Takahashi JS; Schibler U. 2007. System-driven and oscillator-dependent circadian transcription in mice with a conditionally active liver clock. PLoS Biol 5(2):e34. PubMed: 17298173 MGI: J:141590
Kota J; Chivukula RR; O'Donnell KA; Wentzel EA; Montgomery CL; Hwang HW; Chang TC; Vivekanandan P; Torbenson M; Clark KR; Mendell JR; Mendell JT. 2009. Therapeutic microRNA delivery suppresses tumorigenesis in a murine liver cancer model. Cell 137(6):1005-17. PubMed: 19524505 MGI: J:151413
Kress TR; Pellanda P; Pellegrinet L; Bianchi V; Nicoli P; Doni M; Recordati C; Bianchi S; Rotta L; Capra T; Rava M; Verrecchia A; Radaelli E; Littlewood TD; Evan GI; Amati B. 2016. Identification of MYC-Dependent Transcriptional Programs in Oncogene-Addicted Liver Tumors. Cancer Res 76(12):3463-72. PubMed: 27197165 MGI: J:232786
Li Z; Kelly L; Heiman M; Greengard P; Friedman JM. 2015. Hypothalamic Amylin Acts in Concert with Leptin to Regulate Food Intake. Cell Metab 22(6):1059-67. PubMed: 26655697 MGI: J:235897
Ma C; Kesarwala AH; Eggert T; Medina-Echeverz J; Kleiner DE; Jin P; Stroncek DF; Terabe M; Kapoor V; ElGindi M; Han M; Thornton AM; Zhang H; Egger M; Luo J; Felsher DW; McVicar DW; Weber A; Heikenwalder M; Greten TF. 2016. NAFLD causes selective CD4(+) T lymphocyte loss and promotes hepatocarcinogenesis. Nature 531(7593):253-7. PubMed: 26934227 MGI: J:229665
May D; Djonov V; Zamir G; Bala M; Safadi R; Sklair-Levy M; Keshet E. 2011. A transgenic model for conditional induction and rescue of portal hypertension reveals a role of VEGF-mediated regulation of sinusoidal fenestrations. PLoS One 6(7):e21478. PubMed: 21779329 MGI: J:176264
Ney JT; Schmidt T; Kurts C; Zhou Q; Eckert D; Felsher DW; Schorle H; Knolle P; Tuting T; Barchet W; Buttner R; Limmer A; Gutgemann I. 2009. Autochthonous liver tumors induce systemic T cell tolerance associated with T cell receptor down-modulation. Hepatology 49(2):471-81. PubMed: 19105207 MGI: J:186226
Nguyen LH; Robinton DA; Seligson MT; Wu L; Li L; Rakheja D; Comerford SA; Ramezani S; Sun X; Parikh MS; Yang EH; Powers JT; Shinoda G; Shah SP; Hammer RE; Daley GQ; Zhu H. 2014. Lin28b is sufficient to drive liver cancer and necessary for its maintenance in murine models. Cancer Cell 26(2):248-61. PubMed: 25117712 MGI: J:214596
O'Donnell KA; Keng VW; York B; Reineke EL; Seo D; Fan D; Silverstein KA; Schrum CT; Xie WR; Mularoni L; Wheelan SJ; Torbenson MS; O'Malley BW; Largaespada DA; Boeke JD. 2012. A Sleeping Beauty mutagenesis screen reveals a tumor suppressor role for Ncoa2/Src-2 in liver cancer. Proc Natl Acad Sci U S A 109(21):E1377-86. PubMed: 22556267 MGI: J:184782
Ribeiro AC; Ceccarini G; Dupre C; Friedman JM; Pfaff DW; Mark AL. 2011. Contrasting effects of leptin on food anticipatory and total locomotor activity. PLoS One 6(8):e23364. PubMed: 21853117 MGI: J:176508
Roy CN; Mak HH; Akpan I; Losyev G; Zurakowski D; Andrews NC. 2007. Hepcidin antimicrobial peptide transgenic mice exhibit features of the anemia of inflammation. Blood 109(9):4038-44. PubMed: 17218383 MGI: J:145326
Saddic LA; Wirt S; Vogel H; Felsher DW; Sage J. 2011. Functional Interactions between Retinoblastoma and c-MYC in a Mouse Model of Hepatocellular Carcinoma. PLoS One 6(5):e19758. PubMed: 21573126 MGI: J:172430
Schonig K; Kentner D; Gossen M; Baldinger T; Miao J; Welzel K; Vente A; Bartsch D; Bujard H. 2011. Development of a BAC vector for integration-independent and tight regulation of transgenes in rodents via the Tet system. Transgenic Res 20(3):709-20. PubMed: 20640885 MGI: J:172622
Shachaf CM; Kopelman AM; Arvanitis C; Karlsson A; Beer S; Mandl S; Bachmann MH; Borowsky AD; Ruebner B; Cardiff RD; Yang Q; Bishop JM; Contag CH; Felsher DW. 2004. MYC inactivation uncovers pluripotent differentiation and tumour dormancy in hepatocellular cancer. Nature 431(7012):1112-7. PubMed: 15475948 MGI: J:93899
Skvorak KJ; Hager EJ; Arning E; Bottiglieri T; Paul HS; Strom SC; Homanics GE; Sun Q; Jansen EE; Jakobs C; Zinnanti WJ; Gibson KM. 2009. Hepatocyte transplantation (HTx) corrects selected neurometabolic abnormalities in murine intermediate maple syrup urine disease (iMSUD). Biochim Biophys Acta 1792(10):1004-10. PubMed: 19699299 MGI: J:164850
Sun Y; Quinn B; Xu YH; Leonova T; Witte DP; Grabowski GA. 2006. Conditional expression of human acid beta-glucosidase improves the visceral phenotype in a Gaucher disease mouse model. J Lipid Res 47(10):2161-70. PubMed: 16861620 MGI: J:116525
Tao GZ; Lehwald N; Jang KY; Baek J; Xu B; Omary MB; Sylvester KG. 2013. Wnt/beta-catenin signaling protects mouse liver against oxidative stress-induced apoptosis through the inhibition of forkhead transcription factor FoxO3. J Biol Chem 288(24):17214-24. PubMed: 23620592 MGI: J:199603
Tilli MT; Furth PA. 2003. Conditional mouse models demonstrate oncogene-dependent differences in tumor maintenance and recurrence. Breast Cancer Res 5(4):202-5. PubMed: 12817992 MGI: J:84503
Tward AD; Jones KD; Yant S; Cheung ST; Fan ST; Chen X; Kay MA; Wang R; Bishop JM. 2007. Distinct pathways of genomic progression to benign and malignant tumors of the liver. Proc Natl Acad Sci U S A 104(37):14771-14776. PubMed: 17785413 MGI: J:124960
Wang R; Ferrell LD; Faouzi S; Maher JJ; Bishop JM. 2001. Activation of the met receptor by cell attachment induces and sustains hepatocellular carcinomas in transgenic mice. J Cell Biol 153(5):1023-34. PubMed: 11381087 MGI: J:69731
Woodard LE; Keravala A; Jung WE; Wapinski OL; Yang Q; Felsher DW; Calos MP. 2010. Impact of hydrodynamic injection and phiC31 integrase on tumor latency in a mouse model of MYC-induced hepatocellular carcinoma. PLoS One 5(6):e11367. PubMed: 20614008 MGI: J:161998
Xiang Y; Stine ZE; Xia J; Lu Y; O'Connor RS; Altman BJ; Hsieh AL; Gouw AM; Thomas AG; Gao P; Sun L; Song L; Yan B; Slusher BS; Zhuo J; Ooi LL; Lee CG; Mancuso A; McCallion AS; Le A; Milone MC; Rayport S; Felsher DW; Dang CV. 2015. Targeted inhibition of tumor-specific glutaminase diminishes cell-autonomous tumorigenesis. J Clin Invest 125(6):2293-306. PubMed: 25915584 MGI: J:222972
Yang YA; Zhang GM; Feigenbaum L; Zhang YE. 2006. Smad3 reduces susceptibility to hepatocarcinoma by sensitizing hepatocytes to apoptosis through downregulation of Bcl-2. Cancer Cell 9(6):445-57. PubMed: 16766264 MGI: J:110133

Additional - Tg(tetO-DBT)A1Geh related

Skvorak KJ; Hager EJ; Arning E; Bottiglieri T; Paul HS; Strom SC; Homanics GE; Sun Q; Jansen EE; Jakobs C; Zinnanti WJ; Gibson KM. 2009. Hepatocyte transplantation (HTx) corrects selected neurometabolic abnormalities in murine intermediate maple syrup urine disease (iMSUD). Biochim Biophys Acta 1792(10):1004-10. PubMed: 19699299 MGI: J:164850

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Also Known As: MSUD murine model

Donating Investigator

Genetic overview

Research Applications

Base Price

Detailed Description

Development

Expression Data

Control Suggestions

Additional Information

Selected References

Dbttm1Geh

Allele Symbol: Dbttm1Geh

Tg(Cebpb-tTA)5Bjd

Allele Symbol: Tg(Cebpb-tTA)5Bjd

Tg(tetO-DBT)A1Geh

Allele Symbol: Tg(tetO-DBT)A1Geh

Disease Terms

Research Areas By Genotype

This mouse can be used to support research in many areas including:

Mammalian Phenotype Terms by Genotype

Genotype: Dbttm1Geh/Dbttm1Geh Tg(tetO-DBT)A1Geh/0 Tg(Cebpb-tTA)5Bjd/0involves: 129S1/Sv * 129X1/SvJ * C57BL/6J * FVB * NMRI

mortality/aging

homeostasis/metabolism phenotype

References

Additional - Dbttm1Geh related

Additional - Tg(Cebpb-tTA)5Bjd related

Additional - Tg(tetO-DBT)A1Geh related

Genotyping Protocols

Breeding Considerations

Animal Health Reports

Production of mice from cryopreserved embryos or sperm occurs in a maximum barrier room, G200

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Dbt^tm1Geh

Allele Symbol: Dbt^tm1Geh

Genotype: Dbt^tm1Geh/Dbt^tm1Geh Tg(tetO-DBT)A1Geh/0 Tg(Cebpb-tTA)5Bjd/0
involves: 129S1/Sv * 129X1/SvJ * C57BL/6J * FVB * NMRI

Additional - Dbt^tm1Geh related