ATP-Binding Cassette Sub-Family D Member 3 (ABCD3) Antibody

Background

ABCD3, also known as PMP70, is a critical peroxisomal transporter involved in the import of a broad range of substrates, including bile acid intermediates, branched-chain fatty acids, and very-long-chain fatty acids (VLCFAs). It facilitates the transport of these molecules into peroxisomes, where they undergo β-oxidation or other metabolic processes. ABCD3 is highly expressed in tissues such as the liver, kidneys, and adrenal glands, reflecting its role in maintaining lipid homeostasis and detoxification. Mutations or dysregulation of ABCD3 can result in impaired peroxisomal function, contributing to disorders such as Zellweger spectrum disorders, characterized by defects in peroxisomal metabolism. ABCD3’s role in lipid transport underscores its significance in metabolic and developmental processes.