HLCS sirna

HLCS siRNA (Human)

Cat. Num. AAA8225852

• Gene Names: HLCS; HCS
• Reactivity: Human
• Applications: RNA Interference (RNAi)
• Purity: > 97%
• Synonyms: HLCS; HLCS siRNA (Human); Biotin--protein ligase; Biotin apo-protein ligase) ligase; Biotin--[propionyl-CoA-carboxylase [ATP-hydrolyzing]] ligase; Holocarboxylase synthetase; HCS); Biotin--[methylcrotonoyl-CoA-carboxylase] ligase; Biotin--[acetyl-CoA-carboxylase] ligase ]; HLCS sirna

• Host: Synthetic
• Reactivity: Human
• Specificity: HLCS siRNA (Human) is a target-specific 19-23 nt siRNA oligo duplexes designed to knock down gene expression.
• Purity/Purification: > 97%
• Form/Format: Lyophilized powder
• Sequence Length: 726

• Applicable Applications for HLCS sirna: RNA Interference (RNAi)
• Quality Control: Oligonucleotide synthesis is monitored base by base through trityl analysis to ensure appropriate coupling efficiency. The oligo is subsequently purified by affinity-solid phase extraction. The annealed RNA duplex is further analyzed by mass spectrometry to verify the exact composition of the duplex. Each lot is compared to the previous lot by mass spectrometry to ensure maximum lot-to-lot consistency.
• Directions for Use: We recommends transfection with 100 nM siRNA 48 to 72 hours prior to cell lysis. Before resuspending, briefly centrifuge the tube to ensure the lyophilized siRNA is at the bottom of the tube. Resuspend the siRNA oligos to an appropriate concentration with DEPC water. For each vial, suitable for 250 transfections in 24 well plate (20 pmol for each well).
• Components: We offer pre-designed sets of 3 different target-specific siRNA oligo duplexes of human HLCS gene. Each vial contains 5 nmol of lyophilized siRNA. The duplexes can be transfected individually or pooled together to achieve knockdown of the target gene, which is most commonly assessed by qPCR or western blot. Our siRNA oligos are also chemically modified (2'-OMe) at no extra charge for increased stability and enhanced knockdown in vitro and in vivo.
• Preparation and Storage: Shipped at 4 degree C. Store at -20 degree C for one year.

Related Product Information for HLCS sirna

siRNA to inhibit HLCS expression using RNA interference

NCBI and Uniprot Product Information

• NCBI GI #: 46255045
• NCBI GeneID: 3141
• NCBI Accession #: NP_000402.3
• NCBI GenBank Nucleotide #: NM_000411.6
• UniProt Accession #: P50747; Q99451; B2RAH1; D3DSG6
• Molecular Weight: 80,760 Da
• NCBI Official Full Name: biotin--protein ligase
• NCBI Official 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
• UniProt Protein Name: Biotin--protein ligase
• Protein Family: Biotin--protein ligase
• UniProt Gene Name: HLCS
• UniProt Synonym Gene Names: HCS
• UniProt Entry Name: BPL1_HUMAN

NCBI Description

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 Description

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

Chromosomal Location of Human Ortholog: 21q22.13

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

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

Biological Process: cell proliferation; vitamin metabolic process; histone modification; protein amino acid biotinylation; water-soluble vitamin metabolic process; biotin metabolic process

Disease: Holocarboxylase Synthetase Deficiency