MT-ND1 is a gene of the mitochondrial genome coding for the NADH-ubiquinone oxidoreductase chain 1 (ND1) protein. [5] 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. [6]

Mar 30, 2025 · MT-ND1 (Mitochondrially Encoded NADH:Ubiquinone Oxidoreductase Core Subunit 1) is a Protein Coding gene. Diseases associated with MT-ND1 include Leber Hereditary Optic Neuropathy, Modifier Of and Mitochondrial Myopathy, Encephalopathy, Lactic Acidosis, And Stroke-Like Episodes.

The MT-ND1 gene provides instructions for making a protein called NADH dehydrogenase 1. This protein is part of a large enzyme complex known as complex I, which is active in mitochondria.

Mutations affecting MT-ND1 have been described both in homoplasmic and heteroplasmic form: for example, Rafiq J et al. described a family carrying a new homoplasmic mtDNA m.4087A>G mutation in the ND1 gene (MT-ND1), associated with isolated myopathy, recurrent episodes of myoglobinuria, and rhabdomyolysis ; conversely, Gorman GS et al. reported ...

Apr 7, 2025 · Enables NADH dehydrogenase (ubiquinone) activity. Involved in mitochondrial electron transport, NADH to ubiquinone and mitochondrial respiratory chain complex I assembly. Located in mitochondrial membrane.

Feb 8, 2025 · Enables NADH dehydrogenase (ubiquinone) activity. Involved in mitochondrial electron transport, NADH to ubiquinone and mitochondrial respiratory chain complex I assembly. Located in mitochondrial membrane.

Mitochondrial respiratory chain complex I is an important component of the oxidative respiratory chain, with the mitochondrially encoded NADH:ubiquinone oxidoreductase core subunit 1 (MT-ND1) being one of the core subunits. MT-ND1 plays a role in ...

MTND1 is encoded by the guanine-rich heavy (H) strand of the mtDNA between nucleotide pairs (nps) 3307 and 4262 (Anderson et al., 1981; Wallace et al., 1994). It is maternally inherited along with the mtDNA (Giles et al., 1980; Case and Wallace, 1981). The MTND1 gene encompasses 955 nps of continuous coding sequence.

The mitochondrially encoded NADH: ubiquinone oxidoreductase core subunit 1 (MT-ND1) is the largest subunit coded by mitochondria of complex I. The present study summarizes the structure and biological function of MT-ND1.

Genetically heterogeneous disorder, characterized by episodic vomiting, seizures, and recurrent cerebral insults resembling strokes and causing hemiparesis, hemianopsia, or cortical blindness.