Rabbit anti-Mouse 3-Hydroxyisobutyryl Coenzyme A Hydrolase Polyclonal Antibody | anti-HIBCH antibody
Polyclonal Antibody to 3-Hydroxyisobutyryl Coenzyme A Hydrolase (HIBCH)
Immunohistochemistry in formalin fixed frozen section: 1:50-500
Enzyme-linked Immunosorbent Assay: 1:100-1:5000
Usage: 10uL per well when 3,3'-Diaminobenzidine(DAB) as the substrate.
5uL per well when used in enhanced chemilumescent (ECL).
Note: The quality control is specifically manufactured as the positive control. Not used for other purposes.
Loading Buffer: 100mM Tris(pH8.8), 2% SDS, 200mM NaCl, 50% glycerol,BPB 0.01%, NaN3 0.02%.
Western Blot (WB)
(Western Blot: Sample: Mouse Lung lysate; Primary Ab: 1ug/ml Rabbit Anti-Mouse HIBCH Antibody Second Ab: 0.2ug/mL HRP-Linked Caprine Anti-Rabbit IgG Polyclonal Antibody )
NCBI and Uniprot Product Information
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: Amino Acid Metabolism - valine, leucine and isoleucine degradation; Carbohydrate Metabolism - propanoate; EC 3.1.2.4; Hydrolase; Mitochondrial; Other Amino Acids Metabolism - beta-alanine
Chromosomal Location of Human Ortholog: 1|1 C1.1
Cellular Component: extracellular exosome; mitochondrion
Molecular Function: 3-hydroxyisobutyryl-CoA hydrolase activity; hydrolase activity
Biological Process: branched-chain amino acid catabolic process
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Product Notes
The HIBCH hibch (Catalog #AAA2033922) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The Polyclonal Antibody to 3-Hydroxyisobutyryl Coenzyme A Hydrolase (HIBCH) reacts with Mouse and may cross-react with other species as described in the data sheet. AAA Biotech's 3-Hydroxyisobutyryl Coenzyme A Hydrolase can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB), Immunocytochemistry (ICC), Immunohistochemistry (IHC) Formalin/Paraffin, ELISA (EIA). Western blotting: 1:50-400 Immunohistochemistry in formalin fixed frozen section: 1:50-500 Enzyme-linked Immunosorbent Assay: 1:100-1:5000. 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. It is sometimes possible for the material contained within the vial of "3-Hydroxyisobutyryl Coenzyme A Hydrolase, 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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