Lipoic Acid Synthetase (LIAS)

LIAS localizes in mitochondrion and plays an important role in alpha-(+)-lipoic acid synthesis. It may also function in the sulfur insertion chemistry in lipoate biosynthesis. Alternative splicing occurs at this locus and two transcript variants encoding distinct isoforms have been identified.The deduced 373-amino acid protein has a calculated molecular mass of about 42 kD. The N-terminal 26 amino acids encode a potential mitochondrial targeting presequence that, upon removal, would result in a deduced mature protein of 347 amino acids with a molecular mass of about 39 kD. Northern blot analysis detected expression of a 1.8-kb message predominantly in heart, testis, and liver. Transfection of fluorescence-tagged Lias into CHO cells revealed intracellular localization that overlapped with a mitochondrial marker.