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NADH dehydrogenase subunit-1 (NADH ubiquinone oxidoreductase chain-1, MT-ND1, MTND1, NADH1, ND1)
Function:
- component of NADH dehydrogenase (mitochondrial complex 1)
NADH + ubiquinone NAD+ + ubiquinol
Structure: belongs to the complex 1 subunit 1 family
Compartment: mitochondria inner membrane
Pathology:
- defects in MT-ND1 are a cause of
a) Leber hereditary optic neuropathy
b) MELAS syndrome
c) mitochondrial susceptibility to Alzheimer disease
d) non-insulin-dependent diabetes mellitus (NIDDM)
Related
NADH dehydrogenase subunit-1 gene
General
flavoprotein
NADH dehydrogenase subunit
Properties
SIZE: entity length = 318 aa
MW = 36 kD
MOTIF: membrane region
cofactor-binding site
COFACTOR-BOUND: flavin
cofactor-binding site
FOR-BINDING-OF: ubiquinone
Database Correlations
OMIM correlations
UniProt P03886
Pfam PF00146
Kegg hsa:4535
ENZYME 1.6.5.3
References
- UniProt :accession P03886
- GeneReviews
https://www.genecards.org/cgi-bin/carddisp.pl?gene=MT-ND1
Component-of
NADH dehydrogenase; respiratory chain complex-I; NADH CoQ reductase;NADH-ubiquinone oxidoreductase