Gene family: NADH dehydrogenase (ubiquinone); complex I of the mitochondrial respiratory chain; (NDUFA1 NDUFA2 NDUFA3 NDUFA4 NDUFA5 NDUFA5P1 NDUFA6 NDUFA7 NDUFA8 NDUFA9 NDUFA10 NDUFAB1 NDUFB1 NDUFB2 NDUFB3 NDUFB4 NDUFB5 NDUFB6 NDUFB7 NDUFB8 NDUFB9 NDUFB10 NDUFC1 NDUFC2 NDUFS1 NDUFS2 NDUFS2L NDUFS3 NDUFS4 NDUFS5 NDUFS6 NDUFS7 NDUFS8 NDUFV1 NDUFV2 NDUFV2P1 NDUFV3)

COM

Adenosine triphosphate (ATP) is generated via oxidative phosphorylation in the mitochondria of almost all cells. The protein components of the process, 4 respiratory chain complexes (I-IV) and an ATP synthase, are encoded by genes in both the mitochondrial and nuclear genomes. Of the 4 respiratory chain complexes, complex I is the largest at 900 kD and appears to be the most commonly affected in adult human mitochondrial diseases (Dunbar-1997)."

REF

FAG "Dunbar DR &: CCG, 78, N1, 21-24, 1997