Recombinant Human NADH-ubiquinone oxidoreductase chain 1 (MT-ND1), Truncated

Uniprot Id

P03886

Target Species

Human

Target Name

MT-ND1

Target Full Name

NADH-ubiquinone oxidoreductase chain 1

Target Function

Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) which catalyzes electron transfer from NADH through the respiratory chain, using ubiquinone as an electron acceptor. Essential for the catalytic activity and assembly of complex I.

Target Involvement

Leber hereditary optic neuropathy (LHON); Mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes syndrome (MELAS); Alzheimer disease mitochondrial (AD-MT); Diabetes mellitus, non-insulin-dependent (NIDDM); Mitochondrial complex I deficiency (MT-C1D)

Target Subcellular Location

Mitochondrion inner membrane; Multi-pass membrane protein.

Target Protein Families

Complex I subunit 1 family

Target Synonyms

Complex I; subunit ND1; Mitochondrially encoded NADH dehydrogenase 1; MT-ND1; MTND1 ; NAD1 ; NADH dehydrogenase subunit 1 (complex I) ; NADH dehydrogenase subunit 1; NADH-ubiquinone oxidoreductase chain 1; NADH-ubiquinone oxidoreductase; subunit ND1; NADH1 ; ND1 ; NU1M_HUMAN

Target Background

Enables NADH dehydrogenase (ubiquinone) activity. Involved in mitochondrial electron transport, NADH to ubiquinone and mitochondrial respiratory chain complex I assembly. Located in mitochondrial membrane. Part of mitochondrial respiratory chain complex I. Implicated in several diseases, including MELAS syndrome; neurodegenerative disease (multiple); optic nerve disease (multiple); toxic shock syndrome; and type 2 diabetes mellitus. Biomarker of Alzheimer's disease; Parkinson's disease; and multiple sclerosis.