Mitochondrially encoded NADH dehydrogenase 3 is encoded by the mitochondrial gene MT-ND3 and is a core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase. Located in the mitochondrial inner membrane, Complex I is the largest of the five respiratory complexes and catalyzes electron transfer from NADH to ubiquinone. The mitochondrially encoded subunits of Complex I, including MT-ND3, are the most hydrophobic subunits and form the core of the transmembrane region. Pathogenic variants of the MT-ND3 gene are known to cause mitochondrial complex I deficiency and may lead to a wide range of clinical disorders, including Leigh syndrome, Leber hereditary optic neuropathy, and encephalopathy.
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