Rabbit anti-human 3-hydroxyisobutyryl-Coenzyme A hydrolase polyclonal Antibody | MyBioSource MBS713347 product information

product summary

company name :

MyBioSource

product type :

antibody

product name :

Rabbit anti-human 3-hydroxyisobutyryl-Coenzyme A hydrolase polyclonal Antibody

catalog :

MBS713347

quantity :

0.05 mL

price :

375 USD

clonality :

polyclonal

host :

rabbit

conjugate :

nonconjugated

reactivity :

human, mouse, rat

application :

western blot, ELISA, immunohistochemistry, enzyme immunoassay

more info or order :

MyBioSource product webpage

product information

catalog number :

MBS713347

products type :

Antibody

products full name :

Rabbit anti-human 3-hydroxyisobutyryl-Coenzyme A hydrolase polyclonal Antibody

products short name :

3-hydroxyisobutyryl-Coenzyme A hydrolase

products name syn :

3-hydroxyisobutyryl-Coenzyme A hydrolase; HIBCH; HIBYL-COA-H

other names :

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

other gene names :

HIBCH; HIBCH; HIBYLCOAH; HIB-CoA hydrolase; HIBYL-CoA-H

uniprot entry name :

HIBCH_HUMAN

clonality :

Polyclonal

isotype :

IgG

host :

Rabbit

reactivity :

Human,Mouse,Rat; Other species are not tested. Please decide the specificity by homology.

sequence length :

381

purity :

Antigen Affinity Purified

form :

Liquid

storage stability :

PBS with 0.02% sodium azide and 50% glycerol pH 7.3.

tested application :

ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)

app notes :

ELISA: Use at an assay dependent dilution. WB : 1:1000-1:10000 . IHC : 1:20-1:200 (Recommender dilutions). Positive WB detected in : Human brain tissue, human heart tissue, human liver tissue . Positive IHC detected in: Human liver cancer

other info1 :

Immunogen: Human HIBCH. Conjugated: Non-conjugated

other info2 :

Storage Buffer: PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20 degree C, Avoid freeze / thaw cycles.

products description :

Rabbit polyclonal to 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.

ncbi gi num :

3320120

ncbi acc num :

AAC52114.1

ncbi mol weight :

38,012 Da

ncbi pathways :

Branched-chain Amino Acid Catabolism Pathway (106179); Carbon Metabolism Pathway (814926); Carbon Metabolism Pathway (817567); Malonate Semialdehyde Pathway, Propanoyl-CoA = Acetyl-CoA (413478); Malonate Semialdehyde Pathway, Propanoyl-CoA = Acetyl-CoA (468206); Metabolic Pathways (132956); Metabolism Pathway (477135); Metabolism Of Amino Acids And Derivatives Pathway (106169); Propanoate Metabolism Pathway (83004); Propanoate Metabolism Pathway (387)

ncbi summary :

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 summary :

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: Hydrolase; Other Amino Acids Metabolism - beta-alanine; EC 3.1.2.4; Carbohydrate Metabolism - propanoate; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Mitochondrial. 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

size1 :

0.05 mL

price1 :

375 USD

size2 :

0.15 mL

price2 :

715

more info or order :

MyBioSource product webpage