Biochem/physiol Actions

Mitochondrially encoded NADH dehydrogenase 1 (MT-ND1) participates in ubiquinone binding and proton pumping. Mutations in the MT-ND1 gene are implicated in Leber′s hereditary optic neuropathy (LHON), as well as in mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS). Also, some heteroplasmicmutations in the MTND1gene are associated with an overlap syndrome showing the clinical features associated with LHON and MELAS.

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General description

Mitochondrially encoded NADH dehydrogenase 1 (MT-ND1) is a component of mitochondrial membrane respiratory chain nicotinamide adenine dinucleotide (NADH) dehydrogenase (Complex I). It comprises eight transmembrane spanning domains and is a conserved protein placed in the hydrophobic arm of the complex I.

Immunogen

Synthesized peptide derived from internal of human MT-ND1.

Physical form

Rabbit IgG in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.

Specificity

The antibody detects endogenous levels of total MT-ND1 protein.