Western Blot (WB)
(WB Suggested Anti-BPL1 antibody Titration: 1 ug/mLSample Type: Human 786-0 Whole Cell)
Rabbit anti-Human HLCS Polyclonal Antibody | anti-HLCS antibody
HLCS Antibody - C-terminal region
Western Blot (WB)
(WB Suggested Anti-BPL1 antibody Titration: 1 ug/mLSample Type: Human 786-0 Whole Cell)
Western Blot (WB)
(WB Suggested Anti-BPL1 antibody Titration: 1 ug/mLSample Type: Human 786-0 Whole Cell)
Target 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.
NCBI and Uniprot Product Information
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 6.3.4.11; Mitochondrial; EC 6.3.4.15; EC 6.3.4.9; EC 6.3.4.10; Cofactor and Vitamin Metabolism - biotin; Ligase
Chromosomal Location of Human Ortholog: 21q22.13
Cellular Component: nuclear lamina; nuclear matrix; mitochondrion; cytoplasm; chromatin; cytosol
Molecular 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 activity
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
Similar Products
Product Notes
The HLCS hlcs (Catalog #AAA3219885) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The HLCS Antibody - C-terminal region reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's HLCS can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB). Researchers should empirically determine the suitability of the HLCS hlcs for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. The amino acid sequence is listed below: Synthetic peptide located within the following region: MSVAVVEAVR SIPEYQDINL RVKWPNDIYY SDLMKIGGVL VNSTLMGETF. It is sometimes possible for the material contained within the vial of "HLCS, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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