Atypical Kinase COQ8B, Mitochondrial (ADCK4) Antibody

Background

aarF domain-containing kinase 4 (ADCK4), also known as COQ8B, is a mitochondrial protein involved in the biosynthesis and regulation of coenzyme Q10 (CoQ10), an essential molecule in the mitochondrial electron transport chain for ATP production. ADCK4 is particularly important in kidney and podocyte function, and mutations in the ADCK4 gene are associated with CoQ10 deficiency-related nephrotic syndrome, which can lead to kidney dysfunction and proteinuria. Beyond its role in mitochondrial bioenergetics, ADCK4 is thought to stabilize CoQ biosynthetic complexes, ensuring efficient electron transport and reducing oxidative stress. ADCK4 dysfunction is linked to mitochondrial diseases, with CoQ10 supplementation often used as a therapeutic strategy to mitigate symptoms and restore energy metabolism.