MT-ND3

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Map of the human mitochondrial genome

MT-ND3 MT-ND3, also known as Mitochondrially Encoded NADH:Ubiquinone Oxidoreductase Core Subunit 3, is a gene that encodes a subunit of the enzyme complex I, which is a part of the mitochondrial respiratory chain. This gene is located in the mitochondrial DNA and plays a crucial role in the process of oxidative phosphorylation. Structure The MT-ND3 gene is located on the mitochondrial genome and is composed of a specific sequence of nucleotides that encode the ND3 protein. The ND3 protein is a core subunit of complex I, which is responsible for the transfer of electrons from NADH to ubiquinone in the mitochondrial electron transport chain. Function The MT-ND3 gene encodes the ND3 protein, which is essential for the proper functioning of complex I in the mitochondrial respiratory chain. Complex I plays a key role in the generation of ATP, the energy currency of the cell, through oxidative phosphorylation. Mutations in the MT-ND3 gene can lead to mitochondrial dysfunction and various mitochondrial diseases. Clinical Significance Mutations in the MT-ND3 gene have been associated with a range of mitochondrial disorders, including Leber's hereditary optic neuropathy (LHON) and mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes (MELAS). These disorders are characterized by a variety of symptoms affecting different organs and systems in the body. Research Studies investigating the role of the MT-ND3 gene in mitochondrial diseases and cellular metabolism are ongoing. Understanding the function and regulation of this gene may provide insights into the development of potential treatments for mitochondrial disorders. References For further information on MT-ND3 and related topics, please refer to the following resources: - Mitochondrial DNA - Oxidative Phosphorylation - Complex I

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Contributors: Prab R. Tumpati, MD