Monkey 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial (HIBCH) ELISA Kit | HIBCH elisa kit
Monkey 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial (HIBCH) ELISA Kit
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: Other Amino Acids Metabolism - beta-alanine; Carbohydrate Metabolism - propanoate; Hydrolase; Mitochondrial; Amino Acid Metabolism - valine, leucine and isoleucine degradation; EC 3.1.2.4
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 Monkey HIBCH hibch (Catalog #AAA7231339) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA7231339 ELISA Kit recognizes Monkey HIBCH. It is sometimes possible for the material contained within the vial of "3-hydroxyisobutyryl-CoA hydrolase, mitochondrial (HIBCH), ELISA Kit" 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
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