MT-ND1

MT-ND1

Diagram of the human mitochondrial genome, including MT-ND1.

MT-ND1 is a gene located in the mitochondrial DNA of humans. It encodes a subunit of the enzyme NADH dehydrogenase, which is also known as Complex I of the electron transport chain. This enzyme is crucial for the process of oxidative phosphorylation, which is the primary method by which ATP is produced in aerobic respiration.

Structure

The MT-ND1 gene is part of the mitochondrial genome, which is distinct from the nuclear genome. The mitochondrial genome is a circular DNA molecule, and MT-ND1 is one of the 37 genes encoded by this genome. The gene is located at position 3307 to 4262 on the mitochondrial DNA, and it encodes a protein that is 318 amino acids long.

Function

MT-ND1 is a component of Complex I, the first enzyme in the electron transport chain. Complex I is responsible for the transfer of electrons from NADH to ubiquinone, a process that contributes to the generation of a proton gradient across the inner mitochondrial membrane. This proton gradient is used by ATP synthase to produce ATP, the energy currency of the cell.

Clinical Significance

Mutations in the MT-ND1 gene have been associated with several mitochondrial diseases, including Leber's hereditary optic neuropathy (LHON) and Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS). These conditions are characterized by a range of symptoms, often affecting the nervous system and muscles.

Related Pages

• Mitochondrial DNA
• Electron transport chain
• Oxidative phosphorylation
• Leber's hereditary optic neuropathy
• Mitochondrial diseases