HIBCH Human; 3-Hydroxyisobutyryl-CoA Hydrolase Human Recombinant; 3-hydroxyisobutyryl-coenzyme A hydrolase mitochondrial; HIBYL-CoA-H; HIB-CoA hydrolase; EC 3.1.2.4

For Research Use Only. Not for use in diagnostic procedures.

Greater than 90% as determined by SDS-PAGE.

The HIBCH solution (1mg/ml) contains 20mM Tris-HCl buffer (pH 8.0), 200mM Nacl, 1mM DTT and 10% glycerol.

Store at 4°C if entire vial will be used within 2-4 weeks.
Store, frozen at -20°C for longer periods of time.
For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA).

Avoid multiple freeze-thaw cycles.

Small volumes of HIBCH recombinant protein 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.

HIBCH Recombinant produced in E. coli is a single polypeptide chain containing 379 amino acids (33-386) and having a molecular mass of 42.1kDa.
HIBCH is fused to a 25 amino acid His-tag at N-terminus & purified by proprietary chromatographic techniques.

ENZYMES; Enzymes; Hydrolase

3-hydroxyisobutyryl-CoA hydrolase

3-hydroxyisobutyryl-CoA hydrolase, mitochondrial; 3-hydroxyisobutyryl-Coenzyme A hydrolase; HIB-CoA hydrolase; HIBYL-CoA-H

3-hydroxyisobutyryl-CoA hydrolase, mitochondrial

HIB-CoA hydrolase; HIBYL-CoA-H??[Similar Products]

HIBCH_HUMAN

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]

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

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