Biotin--Protein Ligase (HLCS), ELISA Kit

For Research Use Only. Not for use in diagnostic procedures.

Store all reagents at 2-8 degree C

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Small volumes of HLCS elisa kit vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

MBS9395658 is a ready-to-use microwell, strip-or-full plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the Biotin--Protein Ligase (HLCS) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing HLCS. The ELISA analytical biochemical technique of the MBS9395658 kit is based on HLCS antibody-HLCS antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect HLCS antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, HLCS. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

80,760 Da

holocarboxylase synthetase

biotin--protein ligase

Biotin--protein ligase

HCS  [Similar Products]

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. Defects in this gene are the cause of holocarboxylase synthetase deficiency. Multiple alternatively spliced variants, encoding the same protein, have been identified.[provided by RefSeq, Jun 2011]

HLCS: Post-translational modification of specific protein by attachment of biotin. Acts on various carboxylases such as acetyl- CoA-carboxylase, pyruvate carboxylase, propionyl CoA carboxylase, and 3-methylcrotonyl CoA carboxylase. Defects in HLCS are the cause of holocarboxylase synthetase deficiency (HLCS deficiency); also known as biotin-responsive multiple carboxylase deficiency. HLCS deficiency is a neonatal form of multiple carboxylase deficiency, an autosomal recessive disorder characterized by metabolic ketoacidosis, hyperammonemia, excretion of abnormal organic acid metabolites and dermatitis. Clinical and biochemical symptoms improve dramatically with administration of biotin. Belongs to the biotin--protein ligase family.

Protein type: Cofactor and Vitamin Metabolism - biotin; EC 6.3.4.10; EC 6.3.4.11; EC 6.3.4.15; EC 6.3.4.9; Ligase; Mitochondrial

Chromosomal Location of Human Ortholog: 21q22.13

Cellular Component: chromatin; cytoplasm; cytosol; nuclear lamina; nuclear matrix

Molecular Function: biotin binding; biotin-[propionyl-CoA-carboxylase (ATP-hydrolyzing)] ligase activity; biotin-protein ligase activity; enzyme binding; protein binding

Biological Process: biotin metabolic process; cell proliferation; histone modification; protein amino acid biotinylation

Disease: Holocarboxylase Synthetase Deficiency

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