Name: HLCS Antibody (PACO59740)
SKU: PACO59740
Availability: OutOfStock

Antibody Name:	HLCS Antibody (PACO59740)
Antibody SKU:	PACO59740
Size:	50ug
Host Species:	Rabbit
Tested Applications:	ELISA, IHC
Recommended Dilutions:	ELISA:1:2000-1:10000, IHC:1:200-1:500
Species Reactivity:	Human
Immunogen:	Recombinant Human Biotin--protein ligase protein (603-714AA)

Form:	Liquid
Storage Buffer:	Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
Purification Method:	>95%, Protein G purified
Clonality:	Polyclonal
Isotype:	IgG
Conjugate:	Non-conjugated

IHC image of PACO59740 diluted at 1:200 and staining in paraffin-embedded human endometrial cancer performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.

IHC image of PACO59740 diluted at 1:200 and staining in paraffin-embedded human prostate tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.

Background:	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.
Synonyms:	Biotin--protein ligase (EC 6.3.4) (Biotin apo-protein ligase) [Includes: Biotin--[methylmalonyl-CoA-carboxytransferase] ligase (EC 6.3.4.9); Biotin--[propionyl-CoA-carboxylase [ATP-hydrolyzing]] ligase (EC 6.3.4.10) (Holocarboxylase synthetase) (HCS); Biotin--[methylcrotonoyl-CoA-carboxylase] ligase (EC 6.3.4.11); Biotin--[acetyl-CoA-carboxylase] ligase (EC 6.3.4.15)], HLCS

UniProt Protein Function:	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: EC 6.3.4.11; Mitochondrial; EC 6.3.4.15; EC 6.3.4.9; EC 6.3.4.10; Cofactor and Vitamin Metabolism - biotin; LigaseChromosomal Location of Human Ortholog: 21q22.13Cellular Component: nuclear lamina; nuclear matrix; mitochondrion; cytoplasm; chromatin; cytosolMolecular Function: protein binding; biotin-[propionyl-CoA-carboxylase (ATP-hydrolyzing)] ligase activity; enzyme binding; protein homodimerization activity; biotin-[acetyl-CoA-carboxylase] ligase activity; biotin-[methylmalonyl-CoA-carboxytransferase] ligase activity; biotin-protein ligase activity; biotin binding; ATP binding; biotin-[methylcrotonoyl-CoA-carboxylase] ligase activityBiological Process: cell proliferation; vitamin metabolic process; histone modification; protein amino acid biotinylation; water-soluble vitamin metabolic process; biotin metabolic processDisease: Holocarboxylase Synthetase Deficiency
UniProt Protein Details:
NCBI Summary:	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]
UniProt Code:	P50747
NCBI GenInfo Identifier:	1705499
NCBI Gene ID:	3141
NCBI Accession:	P50747.1
UniProt Secondary Accession:	P50747,Q99451, B2RAH1, D3DSG6
UniProt Related Accession:	P50747
Molecular Weight:	726
NCBI Full Name:	Biotin--protein ligase
NCBI Synonym Full Names:	holocarboxylase synthetase (biotin-(proprionyl-CoA-carboxylase (ATP-hydrolysing)) ligase)
NCBI Official Symbol:	HLCS
NCBI Official Synonym Symbols:	HCS
NCBI Protein Information:	biotin--protein ligase; biotin apo-protein ligase; biotin--[acetyl-CoA-carboxylase] ligase; biotin--[methylcrotonoyl-CoA-carboxylase] ligase; biotin--[methylmalonyl-CoA-carboxytransferase] ligase; holocarboxylase synthetase (biotin-(proprionyl-Coenzyme A-
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
UniProt Entry Name:	BPL1_HUMAN