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COX3 is a multi-pass membrane protein. It belongs to the cytochrome c oxidase subunit 3 family. Defects in COX3 are a cause of Leber hereditary optic neuropathy (LHON) and cytochrome c oxidase deficiency (COX deficiency). Defects in MT-CO3 are also found in mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes (MELAS) syndrome and recurrent myoglobinuria.
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Protein Aliases: 3-methylcrotonyl-CoA carboxylase 2; 3-methylcrotonyl-CoA carboxylase non-biotin-containing subunit; 3-methylcrotonyl-CoA:carbon dioxide ligase subunit beta; biotin carboxylase; MCCase subunit beta; meth; methylcrotonoyl-CoA carboxylase 2 (beta); Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial; methylcrotonoyl-Coenzyme A carboxylase 2 (beta); non-biotin containing subunit of 3-methylcrotonyl-CoA carboxylase; testicular secretory protein Li 29
Gene Aliases: 4930552N12Rik; MCCB; MCCC2
UniProt ID: (Human) Q9HCC0, (Rat) Q5XIT9, (Mouse) Q3ULD5
Entrez Gene ID: (Human) 64087, (Rat) 361884, (Mouse) 78038
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