HIBCH antibody

Principal name

HIBCH antibody

Alternative names for HIBCH antibody

HIB-CoA hydrolase, HIBYL-CoA-H, 3-hydroxyisobutyryl-CoA hydrolase, 3-hydroxyisobutyryl-coenzyme A hydrolase

SwissProt ID

A2VDC2 (Xenla), P28817 (Yeast), Q2HJ73 (Bovin), Q5XIE6 (Rat), Q5ZJ60 (Chick), Q6NVY1 (Human), Q8QZS1 (Mouse)

Gene ID

26275 (HIBCH)

Available reactivities

Hu (Human), Ms (Mouse), Rt (Rat), Can (Canine), Mky (Monkey)

Available hosts

Rabbit, Mouse

Available applications

Western blot / Immunoblot (WB), Immunocytochemistry/Immunofluorescence (ICC/IF), Paraffin Sections (P), Enzyme Immunoassay (E), Flow Cytometry (F), Frozen Sections (C)

Background of HIBCH antibody

HIBCH is a 386 amino acid protein belonging to the enoyl-CoA hydratase/isomerase family. Localizing to the mitochondria, HIBCH is highly expressed in liver and kidney, with lower levels found in heart, muscle and brain. HIBCH hydrolyzes HIBYL-CoA, a saline catabolite, and β-hydroxypropionyl-CoA, an intermediate in the minor pathway involved in the metabolism of proprionate. Existing as two alternatively spliced isoforms, the gene encoding HIBCH maps to human chromosome 2q32.2. Defects to this gene result in HIBCH deficiency (HIBCHD), known alternatively as deficiency of β-hydroxyisobutyryl CoA deacylase or methacrylic aciduria. HIBCHD is characterized by the accumulation of methacrylyl-CoA, a highly reactive compound that undergoes addition reactions with free sulfhydryl groups. Phenotypic symptoms include early deterioration of neurological function, delayed motor skill development and hypotonia.

General readings

Hendrickson,S.L. et al. (2010) PLoS ONE 5 (9), E12862Wu,C. et al. (2007) Proteomics 7 (11), 1775-1785

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Primary Antibodies

Catalog No. Host Iso. Clone Pres. React. Applications  

HIBCH antibody

Anti-HIBCH mouse monoclonal antibody (TA501316) immunofluorescent staining of COS7 cells transiently transfected by pCMV6-ENTRY HIBCH(RC209814). Mouse IgG1 OTI3B10 Purified Hu F, ICC/IF, WB
0.1 ml / €325.00
  OriGene Technologies, Inc.

TrueMAB

HIBCH antibody

Immunohistochemical staining of paraffin-embedded Human Kidney tissue within the normal limits using anti-HIBCH mouse monoclonal antibody. (Heat-induced epitope retrieval by 10mM citric buffer, pH6.0, 100C for 10min, TA501317, Dilution 1:50) Mouse IgG2a OTI3H5 Purified Can, Hu F, P, WB
0.1 ml / €325.00
  OriGene Technologies, Inc.

TrueMAB

HIBCH antibody

Anti-HIBCH mouse monoclonal antibody (TA501318) immunofluorescent staining of COS7 cells transiently transfected by pCMV6-ENTRY HIBCH(RC209814). Mouse IgG1 OTI2A5 Purified Hu F, ICC/IF, WB
0.1 ml / €325.00
  OriGene Technologies, Inc.

TrueMAB

HIBCH antibody

Anti-HIBCH mouse monoclonal antibody (TA501319) immunofluorescent staining of COS7 cells transiently transfected by pCMV6-ENTRY HIBCH(RC209814). Mouse IgG2a OTI1C7 Purified Can, Hu, Rt F, ICC/IF, WB
0.1 ml / €325.00
  OriGene Technologies, Inc.

TrueMAB

HIBCH antibody

Anti-HIBCH mouse monoclonal antibody (TA501320) immunofluorescent staining of COS7 cells transiently transfected by pCMV6-ENTRY HIBCH(RC209814). Mouse IgG2b OTI3E7 Purified Hu, Rt F, ICC/IF, WB
0.1 ml / €325.00
  OriGene Technologies, Inc.

TrueMAB

HIBCH antibody

Anti-HIBCH mouse monoclonal antibody (TA501330) immunofluorescent staining of COS7 cells transiently transfected by pCMV6-ENTRY HIBCH(RC209814). Mouse IgG2b OTI4G1 Purified Can, Hu, Mky, Rt F, ICC/IF, WB
0.1 ml / €379.00
  OriGene Technologies, Inc.

TrueMAB

HIBCH antibody

Anti-HIBCH mouse monoclonal antibody (TA501331) immunofluorescent staining of COS7 cells transiently transfected by pCMV6-ENTRY HIBCH(RC209814). Mouse IgG2b OTI3G1 Purified Hu F, ICC/IF, WB
0.1 ml / €325.00
  OriGene Technologies, Inc.

TrueMAB

HIBCH antibody

Anti-HIBCH mouse monoclonal antibody (TA501332) immunofluorescent staining of COS7 cells transiently transfected by pCMV6-ENTRY HIBCH(RC209814). Mouse IgG1 OTI3F6 Purified Hu F, ICC/IF, WB
0.1 ml / €325.00
  OriGene Technologies, Inc.

TrueMAB

HIBCH antibody

Anti-HIBCH mouse monoclonal antibody (TA501361) immunofluorescent staining of COS7 cells transiently transfected by pCMV6-ENTRY HIBCH(RC209814). Mouse IgG1 OTI5C5 Purified Can, Hu, Rt F, ICC/IF, WB
0.1 ml / €325.00
  OriGene Technologies, Inc.

TrueMAB

HIBCH antibody

Anti-HIBCH mouse monoclonal antibody (TA501387) immunofluorescent staining of COS7 cells transiently transfected by pCMV6-ENTRY HIBCH(RC209814). Mouse IgG1 OTI3D11 Purified Can, Hu, Mky, Rt F, ICC/IF, WB
0.1 ml / €325.00
  OriGene Technologies, Inc.

TrueMAB

HIBCH antibody

Formalin-fixed and paraffin-embedded human hepatocarcinoma tissue reacted with HIBCH antibody (Center), which was peroxidase-conjugated to the secondary antibody, followed by DAB staining. This data demonstrates the use of this antibody for immunohistochemistry; clinical relevance has not been evaluated. Rabbit Ig Purified Hu WB
0.4 ml / €370.00
  Acris Antibodies GmbH

HIBCH antibody

Western Blot: HIBCH Antibody [NBP1-32964] - Western blot analysis of HIBCH on H1299 lysates using NBP1-32964. Rabbit IgG Hu ICC/IF, P, WB
0.1 mg / €420.00
  Novus Biologicals Inc.

Proteins & Growth Factors

Catalog No. Species Pres. Purity Source  

HIBCH (transcript variant 1)

HIBCH Human > 80 %
Preparation: Recombint protein was captured through anti-DDK affinity column followed by conventiol chromatography steps.
Purity Detail: > 80% as determined by SDS-PAGE and Coomassie blue staining.
HEK293 cells

Regular Price: 20 µg / €680.00

Special Price: 20 µg / €398.00

  OriGene Technologies, Inc.

HIBCH (33-386, His-tag)

HIBCH Human Purified > 90 % by SDS - PAGE E. coli
0.25 mg / €940.00
  Acris Antibodies GmbH

HIBCH (33-386, His-tag)

HIBCH Human Purified > 90 % by SDS - PAGE E. coli
50 µg / €370.00
  Acris Antibodies GmbH

HIBCH

HIBCH Human Purified
2 µg / €300.00
  Abnova Taiwan Corp.

16 Item(s)

per page
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