Coenzyme Q-binding protein COQ10 homolog B, mitochondrial (COQ10B)

Coenzyme Q-binding protein COQ10 homolog B, mitochondrial (COQ10B), is a human protein encoded by the *COQ10B* gene and is a homolog of the yeast Coq10 protein. This protein is believed to bind and possibly chaperone coenzyme Q (ubiquinone/CoQ10) within the mitochondrion, facilitating proper localization and function of CoQ10, a critical cofactor in electron transport and antioxidant defense. COQ10B is part of the START domain- and lipid-transfer protein family, indicating a function in mitochondrial lipid trafficking rather than direct enzyme activity or receptor function. In model organisms, its loss impairs respiratory capacity and increases sensitivity to oxidative stress, highlighting a supportive but non-enzymatic, non-receptor role in cellular energy metabolism and oxidative protection[4]. Mutations in homologous proteins can be linked to mitochondrial dysfunction, but there is no evidence that COQ10B itself is a direct therapeutic target in current pharmacology, nor does it interact with drugs such as coenzyme Q10 supplementation[3][4]. Key clarification: - The target name "coenzyme Q10B" or "COQ10B" does not refer directly to coenzyme Q10 or ubiquinone itself, which *is* a therapeutic target, antioxidant, and metabolic cofactor[1][2][5]. Instead, COQ10B is a protein that may bind and help localize or stabilize coenzyme Q10 in mitochondria. - *COQ10B is not considered a canonical therapeutic target* such as an enzyme or receptor, but rather a mitochondrial lipid-binding/chaperone protein. The actual clinical interest and targeting is focused on coenzyme Q10 (ubiquinone), not on the COQ10B protein. Reasons for "is_incorrect: true": - The submitted target is frequently confused with "coenzyme Q10" (ubiquinone, a drug/target), but "COQ10B" is a mitochondrial protein/chaperone and not itself a pharmacological target or therapeutic receptor/enzyme/transporter[4]. - There is no evidence for direct drug targeting, known clinical drugs, or mechanisms of action for COQ10B itself. Distinction: - For structured data regarding mitochondrial coenzyme Q10 as a therapeutic target, see entries for "Coenzyme Q10" or "Ubiquinone".

Other names

Coenzyme Q10 homolog BCOQ10BFLJ13448Coenzyme Q-binding protein COQ10 homolog B, mitochondrial