Western Blot (WB)
(Host: RabbitTarget Name: HIBCHSample Tissue: Liver Tumor lysatesAntibody Dilution: 1.0ug/ml)
Rabbit anti-Human HIBCH Polyclonal Antibody | anti-HIBCH antibody
HIBCH Antibody - C-terminal region
Western Blot (WB)
(Host: RabbitTarget Name: HIBCHSample Tissue: Liver Tumor lysatesAntibody Dilution: 1.0ug/ml)
Western Blot (WB)
(Host: RabbitTarget Name: HIBCHSample Tissue: Liver Tumor lysatesAntibody Dilution: 1.0ug/ml)
NCBI and Uniprot Product Information
NCBI Description
This gene encodes the enzyme responsible for hydrolysis of both HIBYL-CoA and beta-hydroxypropionyl-CoA. Mutations in this gene have been associated with 3-hyroxyisobutyryl-CoA hydrolase deficiency. Alternative splicing results in multiple transcript variants.[provided by RefSeq, May 2010]
Uniprot Description
HIBCH: Hydrolyzes 3-hydroxyisobutyryl-CoA (HIBYL-CoA), a saline catabolite. Has high activity toward isobutyryl-CoA. Could be an isobutyryl-CoA dehydrogenase that functions in valine catabolism. Also hydrolyzes 3-hydroxypropanoyl-CoA. Defects in HIBCH are the cause of HIBCH deficiency (HIBCHD); also known as deficiency of beta- hydroxyisobutyryl CoA deacylase or methacrylic aciduria. The enzyme defect results in accumulation of methacrylyl-CoA, a highly reactive compound, which readily undergoes addition reactions with free sulfhydryl groups. Affected individuals showed delayed development of motor skills, hypotonia, initial poor feeding, and a deterioration in neurological function during first stages of life. Belongs to the enoyl-CoA hydratase/isomerase family. 2 isoforms of the human protein are produced by alternative splicing.
Protein type: Mitochondrial; Other Amino Acids Metabolism - beta-alanine; EC 3.1.2.4; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Carbohydrate Metabolism - propanoate; Hydrolase
Chromosomal Location of Human Ortholog: 2q32.2
Cellular Component: mitochondrial matrix
Molecular Function: 3-hydroxyisobutyryl-CoA hydrolase activity
Biological Process: valine catabolic process; branched chain family amino acid catabolic process
Disease: Beta-hydroxyisobutyryl Coa Deacylase Deficiency
Research Articles on HIBCH
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Product Notes
The HIBCH hibch (Catalog #AAA3220988) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The HIBCH Antibody - C-terminal region reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's HIBCH 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 HIBCH hibch 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: LQEVLTMEYR LSQACMRGHD FHEGVRAVLI DKDQSPKWKP ADLKEVTEED. It is sometimes possible for the material contained within the vial of "HIBCH, 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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