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Human Disease and Mouse Model Detail
Human Disease Mitochondrial Complex I Deficiency
OMIM ID: 252010
Synonyms Mitochondrial Complex Deficiency; Mitochondrial Nadh Dehydrogenase Component of Complex I, Deficiency of; Nadh-Coenzyme Q Reductase Deficiency; NADH:Q(1) Oxidoreductase Deficiency
View all models View ALL (2) mouse models for this human disease.
Genes and
mouse models
Mutations in human and/or mouse homologs are associated with this disease

      *Disease is associated/modeled with this Gene or a homolog. More...
      Mouse Homologs Human Homologs Mouse Models Mouse : Human
Homology Class
     Ndufs4* NDUFS4* View 1 model 1:1 Homology
Ndufs6*, Gm6415 NDUFS6* View 1 model 2:1 Homology
     Foxred1 FOXRED1*   1:1 Homology
Gm4943, Ndufa11 NDUFA11*   2:1 Homology
Ndufa1 NDUFA1*   1:1 Homology
Ndufaf1 NDUFAF1*   1:1 Homology
Ndufaf2 NDUFAF2*   1:1 Homology
Ndufaf3 NDUFAF3*   1:1 Homology
Ndufaf4 NDUFAF4*   1:1 Homology
Ndufaf5 NDUFAF5*   1:1 Homology
Ndufb3 NDUFB3*   1:1 Homology
Ndufb9 NDUFB9*   1:1 Homology
Ndufs1 NDUFS1*   1:1 Homology
Ndufs2 NDUFS2*   1:1 Homology
Ndufs3 NDUFS3*   1:1 Homology
Ndufv1 NDUFV1*   1:1 Homology
Ndufv2 NDUFV2*   1:1 Homology
Nubpl NUBPL*   1:1 Homology
References Disease References using Mouse Models (2)

Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
MGI 5.21
The Jackson Laboratory