Acyl-CoA Dehydrogenase Family Member 9 (ACAD9) Antibody (Biotin)

Background

ACAD9 is a mitochondrial enzyme essential for the β-oxidation of long-chain fatty acids and the assembly of mitochondrial respiratory chain complex I. It functions as a dehydrogenase, catalyzing the first step in the oxidation of long-chain acyl-CoA substrates, generating energy and maintaining lipid homeostasis. ACAD9 is also a critical assembly factor for complex I, facilitating the proper formation and function of this key component of the electron transport chain. Its dual role in fatty acid oxidation and complex I assembly makes ACAD9 indispensable for mitochondrial energy metabolism. Mutations in the ACAD9 gene are associated with complex I deficiency, a severe mitochondrial disorder that can lead to cardiomyopathy, encephalopathy, and metabolic crises. The high expression of ACAD9 in energy-demanding tissues such as the heart, liver, and brain reflects its importance in maintaining mitochondrial function and energy production, positioning it as a vital enzyme in cellular metabolism.