HIBCH, Monoclonal Antibody

Mouse Monoclonal [clone 5C5] (IgG1) to Human HIBCH; Human HIBCH; HIB-CoA hydrolase; HIBYL-CoA-H; HIBYLCOAH

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

Monoclonal

IgG1

5C5

Mouse

Human HIBCH

Protein A/G purified

PBS, pH 7.3, 1% BSA, 50% glycerol, 0.02% sodium azide

0.79 mg/ml (lot specific)

Store at -20 degree C. Minimize freezing and thawing.

Small volumes of anti-HIBCH antibody 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 Antibody, HIB-CoA hydrolase Antibody, HIBYL-CoA-H Antibody, HIBYLCOAH Antibody Description: 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.

Immunohistochemistry (IHC) Paraffin, Immunofluorescence (IF), Western Blot (WB), Flow Cytometry (FC/FACS)

Flow Cytometry (1:100)
IF (1:100)
IHC-P (10 ug/ml)
WB (1:2000)

Anti-HIBCH antibody IHC staining of human liver. Immunohistochemistry of formalin-fixed, paraffin-embedded tissue after heat-induced antigen retrieval. Antibody concentration 10 ug/ml.

Anti-HIBCH mouse monoclonal antibody immunofluorescent staining of COS7 cells transiently transfected by pCMV6-ENTRY HIBCH.

HEK293T cells were transfected with the pCMV6-ENTRY control (Left lane) or pCMV6-ENTRY HIBCH (Right lane) cDNA for 48 hrs and lysed. Equivalent amounts of cell lysates (5 ug per lane) were separated by SDS-PAGE and immunoblotted with anti-HIBCH.

Western blot of extracts (35 ug) from 9 different cell lines by using anti-HIBCH monoclonal antibody.

HEK293T cells transfected with either overexpress plasmid (Red) or empty vector control plasmid (Blue) were immunostained by anti-HIBCH antibody, and then analyzed by flow cytometry.

38,012 Da

3-hydroxyisobutyryl-CoA hydrolase

3-hydroxyisobutyryl-CoA hydrolase, mitochondrial

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

Chromosomal Location of Human Ortholog: 2q32.2

Cellular Component: mitochondrial matrix

Molecular Function: 3-hydroxyisobutyryl-CoA hydrolase activity; hydro-lyase activity

Biological Process: branched chain family amino acid catabolic process; valine catabolic process

Disease: Beta-hydroxyisobutyryl Coa Deacylase Deficiency

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