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COQ4 is a component of the coenzyme Q biosynthesis pathway. Coenzyme Q, an essential component of the electron transport chain, shuttles electrons between complexes I or II to complex III of the mitochondrial transport chain. This protein appears to play a structural role in stabilizing a complex that contains most of the coenzyme Q biosynthesis enzymes. Mutations in this gene are associated with mitochondrial disorders linked to coenzyme Q deficiency. Alternative splicing results in multiple transcript variants.
Gene Name: | coenzyme Q4 |
Synonyms: | COQ4, Coenzyme Q4 homolog (yeast), CGI-92 |
Target Sequences: | NM_016035 NP_057119.2 Q9Y3A0 |
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