NDUFA1 polyclonal antibody

NDUFA1 (NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 1), also known as ZNF183 or MWFE, is one of about 45 subunits comprising Complex I of the oxidative phosphorylation electron transport chain. Consisting of 70 amino acids and expressed predominantly in skeletal muscle and heart, NDUFA1 functions as an accessory subunit of the multi-protein mitochondrial membrane respiratory chain NADH dehydrogenase complex (known as Complex I). Complex I plays an important role in the transfer of electrons from NADH to the respiratory chain, a process that is essential for cellular respiration. As a single-pass membrane protein, NDUFA1 localizes to the matrix side of the mitochondrial inner membrane and is a member of the complex I NDUFA1 subunit family. The gene encoding NDUFA1 maps to human chromosome Xq24 and mouse chromosome X A2. NDUFA1 defects may cause mitochondrial Complex I deficiency, a mitochondrial disorder with wide symptoms ranging from cardiomyopathy, myopathy, liver disease and neurological disorders.