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.
免疫原
Synthetic peptide directed towards the C terminal region of human LIAS
アプリケーション
Rabbit Anti-LIAS antibody is suitable for western blot applications at a concentration of 0.25 μg/ml.
生物化学的/生理学的作用
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.
シーケンス
Synthetic peptide located within the following region: EYITPEKFKYWEKVGNELGFHYTASGPLVRSSYKAGEFFLKNLVAKRKTK
物理的形状
Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.
免責事項
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
American journal of human genetics, 89(6), 792-797 (2011-12-14)
Lipoic acid is an essential prosthetic group of four mitochondrial enzymes involved in the oxidative decarboxylation of pyruvate, α-ketoglutarate, and branched chain amino acids and in the glycine cleavage. Lipoic acid is synthesized stepwise within mitochondria through a process that