COQ8B抗体 新品

The COQ8B antibody is a tool used to detect the COQ8B protein, a member of the ancient ubiquitous CoQ8/ADCK family of mitochondrial kinases. COQ8B, also known as ADCK4. plays a critical role in the biosynthesis of coenzyme Q10 (CoQ10), a lipid-soluble electron carrier in the mitochondrial respiratory chain and a vital antioxidant. Mutations in the COQ8B gene are linked to steroid-resistant nephrotic syndrome (SRNS) and mitochondrial encephalopathy, underscoring its importance in renal and neurological function. The protein is predominantly expressed in the kidney, heart, and skeletal muscle, where it stabilizes the CoQ10 synthesis complex and regulates CoQ10 production. The COQ8B antibody is widely utilized in research to study protein expression, localization, and molecular mechanisms in disease models, particularly in podocyte dysfunction and mitochondrial disorders. It is essential for techniques like Western blotting, immunohistochemistry, and immunofluorescence, aiding in the validation of COQ8B's role in cellular energy metabolism and oxidative stress pathways. This antibody also serves as a diagnostic adjunct in genetic studies to correlate COQ8B mutations with clinical phenotypes, offering insights into potential therapeutic strategies targeting CoQ10 deficiencies.