The Anti-HLCS Antibody, Rabbit Polyclonal from MyBioSource.com is a Rabbit Polyclonal antibody to HLCS. This antibody recognizes Human antigen. The Anti-HLCS Antibody, Rabbit Polyclonal has been shown to work in the following applications: Immunohistochemistry, and Immunohistochemistry - fixed.
Description
HLCS (Holocarboxylase Synthetase) is a Protein Coding gene. This gene encodes an enzyme that catalyzes the binding of biotin to carboxylases and histones. The protein plays an important role in gluconeogenesis, fatty acid synthesis, and branched-chain amino acid catabolism. HLCS belongs to the biotin--protein ligase family. It is widely expressed in the thyroid, prostate, and other tissues. Diseases associated with HLCS include Holocarboxylase Synthetase Deficiency and Multiple Carboxylase Deficiency. HLCS deficiency is a rare autosomal recessive disorder that presents with multiple life-threatening metabolic derangements including metabolic acidosis, ketosis, and hyperammonemia. A majority of HLCS deficiency patients respond to biotin therapy