Application

Rabbit Anti-LIAS antibody is suitable for western blot applications at a concentration of 0.25 µg/ml.

Biochem/physiol Actions

LIAS 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.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.

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General description

Lipoic acid synthetase (LIAS) a mitochondrial protein that catalyzes the synthesis of α-(+)-lipoic acid synthesis. Deficiency of LIAS is linked to glycine elevation, defects in mitochondrial energy metabolism and neonatal-onset epilepsy.Rabbit Anti-LIAS antibody recognizes human, mouse, and rat LIAS antibody.

Immunogen

Synthetic peptide directed towards the C terminal region of human LIAS

Physical form

Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.

Sequence

Synthetic peptide located within the following region: EYITPEKFKYWEKVGNELGFHYTASGPLVRSSYKAGEFFLKNLVAKRKTK