Full Product Name
HLCS Rabbit Polyclonal
Product Gene Name
anti-HLCS antibody
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Species Reactivity
Human, Mouse, Rat
Purity/Purification
>=95% as determined by SDS-PAGE
Immunogen Affinity Purified
Immunogen
Holocarboxylase synthetase (biotin-(proprionyl-Coenzyme A-carboxylase (ATP-hydrolysing)) ligase)
Preparation and Storage
PBS with 0.02% sodium azide and 50% glycerol pH 7.3, -20 degree C for 24 months (Avoid repeated freeze / thaw cycles.)
ISO Certification
Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.
Other Notes
Small volumes of anti-HLCS antibody 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.
Applications Tested/Suitable for anti-HLCS antibody
ELISA (EIA), Western Blot (WB)
NCBI/Uniprot data below describe general gene information for HLCS. It may not necessarily be applicable to this product.
NCBI Accession #
BAB68213.1
[Other Products]
UniProt Related Accession #
Q920N2[Other Products]
Molecular Weight
78,516 Da
NCBI Official Full Name
Hlcs
NCBI Official Synonym Full Names
holocarboxylase synthetase (biotin- [propriony-Coenzyme A-carboxylase (ATP-hydrolysing)] ligase)
NCBI Official Symbol
Hlcs [Similar Products]
NCBI Official Synonym Symbols
D16Jhu34; 410I21.SP6
[Similar Products]
NCBI Protein Information
biotin--protein ligase
UniProt Protein Name
Biotin--protein ligase
UniProt Synonym Protein Names
Biotin apo-protein ligaseIncluding the following 4 domains:Biotin--[methylmalonyl-CoA-carboxytransferase] ligase (EC:6.3.4.9)Biotin--[propionyl-CoA-carboxylase [ATP-hydrolyzing]] ligase (EC:6.3.4.10)Alternative name(s):Holocarboxylase synthetase; HCS
Protein Family
Biotin--protein ligase
UniProt Gene Name
Hlcs [Similar Products]
UniProt Synonym Gene Names
HCS [Similar Products]
UniProt Entry Name
BPL1_MOUSE
UniProt Comments for HLCS
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: Mitochondrial; Cofactor and Vitamin Metabolism - biotin; Ligase; EC 6.3.4.11; EC 6.3.4.10; EC 6.3.4.9; EC 6.3.4.15
Cellular Component: chromatin; cytoplasm; cytosol; mitochondrion; nuclear lamina; nuclear matrix
Molecular Function: ATP binding; biotin binding; biotin-[acetyl-CoA-carboxylase] ligase activity; biotin-[methylcrotonoyl-CoA-carboxylase] ligase activity; biotin-[methylmalonyl-CoA-carboxytransferase] ligase activity; biotin-[propionyl-CoA-carboxylase (ATP-hydrolyzing)] ligase activity; biotin-protein ligase activity; catalytic activity; enzyme binding; ligase activity; lipoate-protein ligase activity; nucleotide binding; protein homodimerization activity; tRNA(Ile)-lysidine synthase
Biological Process: cell proliferation; histone modification; metabolic process; protein amino acid biotinylation; protein modification process
Precautions
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Disclaimer
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