Full Product Name
HLCS siRNA (Human)
Product Synonym Names
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 ]
Product Gene Name
HLCS sirna
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
3D Structure
ModBase 3D Structure for P50747
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
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.
Negative Control
siRNA Negative Control (Catalog# MBS8241404) is a non-targeting 21 nt siRNA recommended as a negative control for experiments using targeted siRNA transfection.
Other Notes
Small volumes of HLCS sirna 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.
Related Product Information for
HLCS sirna
siRNA to inhibit HLCS expression using RNA interference
Applications Tested/Suitable for HLCS sirna
RNA Interference (RNAi)
NCBI/Uniprot data below describe general gene information for HLCS. It may not necessarily be applicable to this product.
NCBI Accession #
NP_000402.3
[Other Products]
NCBI GenBank Nucleotide #
NM_000411.6
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UniProt Primary Accession #
P50747
[Other Products]
UniProt Secondary Accession #
Q99451; B2RAH1; D3DSG6[Other Products]
UniProt Related Accession #
P50747[Other Products]
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 [Similar Products]
NCBI Official Synonym Symbols
HCS
[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_HUMAN
NCBI Summary for HLCS
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 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: 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
Research Articles on HLCS
1. The transcriptional function of HCS was shown by in vitro pull down and in vivo co-immunoprecipitation assays to depend on its interaction with the histone deacetylases HDAC1, HDAC2 and HDAC7
Precautions
All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
Disclaimer
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