Excel File Text File All mouse models of organic acidemia with phenotypic similarity to the human disease
        Disease Term Allelic Composition Genetic Background Reference Phenotypes
      3-methylglutaconic aciduria type 3 Opa3m1Votr/Opa3m1Votr involves: C3H * C57BL/6JCrl J:181670, J:188346 View
3-methylglutaconic aciduria with deafness, encephalopathy, and Leigh-like syndrome Serac1em1Bcgen/Serac1em1Bcgen C57BL/6N-Serac1em1Bcgen J:326672 View
Barth syndrome Gt(ROSA)26Sortm37(H1/tetO-RNAi:Tafazzin)Arte/? Not Specified J:176041 View
Barth syndrome Gt(ROSA)26Sortm37(H1/tetO-RNAi:Tafazzin)Arte/Gt(ROSA)26Sor+ involves: 129S6/SvEvTac * C57BL/6J J:167527 View
Barth syndrome Tafazzinem1Xfa/Y
Tg(myl7.L-cre)1118Tmhn/0		 involves: C57BL/6NCrl * MF1 J:339058 View
glutaric acidemia I Gcdhtm1Dmk/Gcdhtm1Dmk involves: 129S4/SvJae * C57BL/6J J:77874 View
glutaric acidemia I Gcdhtm1Dmk/Gcdhtm1Dmk involves: 129S4/SvJae * C57BL/6N * FVB/N J:256227 View
maple syrup urine disease Bckdhbem1(IMPC)J/Bckdhbem1(IMPC)J CByJ.B6-Bckdhbem1(IMPC)J J:344897 View
maple syrup urine disease Dbttm1Geh/Dbttm1Geh involves: 129S1/Sv * 129X1/SvJ * C57BL/6J J:119973 View
maple syrup urine disease Dbttm1Geh/Dbttm1Geh
Tg(tetO-DBT)525AGeh/0
Tg(Cebpb-tTA)5Bjd/0		 involves: 129S1/Sv * 129X1/SvJ * C57BL/6J * FVB * NMRI J:119973 View
maple syrup urine disease Dbttm1Geh/Dbttm1Geh
Tg(tetO-DBT)A1Geh/0
Tg(Cebpb-tTA)5Bjd/0		 involves: 129S1/Sv * 129X1/SvJ * C57BL/6J * FVB * NMRI J:119973 View
maple syrup urine disease Ppm1ktm1Yiwa/Ppm1ktm1Yiwa involves: 129/Sv * C57BL/6 J:150451 View
methylmalonic acidemia and homocysteinemia cblX type Hcfc1em1Poche/Y C57BL/6J-Hcfc1em1Poche J:317822 View
methylmalonic acidemia due to transcobalamin receptor defect Cd320Gt(CC0426)Wtsi/Cd320Gt(CC0426)Wtsi involves: 129P2/OlaHsd * C57BL/6 J:199961 View
methylmalonic aciduria and homocystinuria type cblC MmachcGt(AZ0348)Wtsi/Mmachc+ involves: 129P2/OlaHsd * 129S6/SvEvTac * C57BL/6 J:212387 View
methylmalonic aciduria due to methylmalonyl-CoA mutase deficiency Mmutem1Cpv/Mmutem1Cpv involves: 129S/SvEv * C57BL/6 * FVB/N J:332892 View
methylmalonic aciduria due to methylmalonyl-CoA mutase deficiency Mmutem2Cpv/Mmutem2Cpv involves: 129S/SvEv * C57BL/6 * FVB/N J:332892 View
methylmalonic aciduria due to methylmalonyl-CoA mutase deficiency Mmuttm1.1Mrb/Mmuttm1.1Mrb involves: C57BL/6 J:237040 View
methylmalonic aciduria due to methylmalonyl-CoA mutase deficiency Mmuttm1Cpv/Mmuttm1Cpv Not Specified J:147338 View
methylmalonic aciduria due to methylmalonyl-CoA mutase deficiency Mmuttm1Cpv/Mmuttm1Cpv
Tg(Alb-Mut)#Cpv/0		 involves: C57BL/6 J:200689 View
methylmalonic aciduria due to methylmalonyl-CoA mutase deficiency Mmuttm1Pai/Mmuttm1.1Mrb involves: 129S1/Sv * C57BL/6 J:237040 View
methylmalonic aciduria due to methylmalonyl-CoA mutase deficiency Mmuttm1Pai/Mmuttm1Pai involves: 129S1/Sv * C57BL/6 J:87081 View
methylmalonic aciduria due to methylmalonyl-CoA mutase deficiency Mmuttm1Pai/Mmuttm1Pai
Tg(MUT)AHlps/0
Tg(MUT*R403X)#Hlps/0		 involves: 129S1/Sv * C57BL/6 J:191879 View
methylmalonic aciduria due to methylmalonyl-CoA mutase deficiency Mmuttm1Pai/Mmuttm1Pai
Tg(MUT*R403X)#Hlps/0		 involves: 129S1/Sv * C57BL/6 J:191879 View
propionic acidemia Pccatm1Tmiy/Pccatm1Tmiy involves: 129P2/OlaHsd * C57BL/6 J:71660 View
propionic acidemia Pccatm1Tmiy/Pccatm1Tmiy
Tg(CAG-PCCA*A138T,-EGFP)#Miab/0		 involves: 129P2/OlaHsd * FVB/N J:286218, J:282292 View
      Barth syndrome Fkbp1atm1Zuk/Fkbp1atm1Zuk either: (involves: 129S7/SvEvBrd) or (involves: 129S7/SvEvBrd * C57BL/6J) J:45536 View
Barth syndrome Mesttm1Masu/Mest+ involves: 129S1/Sv * 129X1/SvJ J:79223 View
maple syrup urine disease Bcat2m1Ytc/Bcat2m1Ytc C57BL/6J-Bcat2m1Ytc J:87589 View
methylmalonic aciduria and homocystinuria type cblC Thap11em1Poche/Thap11em1Poche C57BL/6J-Thap11em1Poche J:317822 View