Aliases for LIAS Gene
External Ids for LIAS Gene
Previous GeneCards Identifiers for LIAS Gene
The protein encoded by this gene belongs to the biotin and lipoic acid synthetases family. It 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. [provided by RefSeq, Jul 2008]
GeneCards Summary for LIAS Gene
LIAS (Lipoic Acid Synthetase) is a Protein Coding gene. Diseases associated with LIAS include Hyperglycinemia, Lactic Acidosis, And Seizures and Lipoic Acid Synthetase Deficiency. Among its related pathways are Metabolism and Glyoxylate metabolism and glycine degradation. Gene Ontology (GO) annotations related to this gene include 4 iron, 4 sulfur cluster binding and lipoate synthase activity.
UniProtKB/Swiss-Prot for LIAS Gene
Catalyzes the radical-mediated insertion of two sulfur atoms into the C-6 and C-8 positions of the octanoyl moiety bound to the lipoyl domains of lipoate-dependent enzymes, thereby converting the octanoylated domains into lipoylated derivatives.