Location of the MT-ND1
gene in the human mitochondrial genome. MT-ND1
is one of the seven NADH dehydrogenase mitochondrial genes (yellow boxes).
NADH-ubiquinone oxidoreductase chain 1 is a protein that in humans is encoded by the mitochondrial gene MT-ND1. The ND1 protein is a subunit of NADH dehydrogenase, which is located in the mitochondrial inner membrane and is the largest of the five complexes of the electron transport chain. Variants of the MT-ND1 gene are associated with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS), Leigh's syndrome (LS), Leber's hereditary optic neuropathy (LHON) and increases in adult BMI.