Overview

  • Product nameAnti-HIBCH antibodySee all HIBCH primary antibodies ...
  • Description
    Rabbit polyclonal to HIBCH
  • Tested applicationsELISA, WB, IHC-P more details
  • Species reactivity
    Reacts with: Human
    Predicted to work with: Mouse, Rat
  • Immunogen

    Recombinant fragment, corresponding to amino acids 98-358 of Human HIBCH (BC005190).

  • Positive control
    • HepG2 cell lysate, Human fetal brain, skeletal muscle, liver, kidney and heart tissue lysates. Human hepatocarcinoma tissue.

Properties

Applications

Our Abpromise guarantee covers the use of ab101672 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

Application Notes
ELISA 1/20000 - 1/80000.
WB 1/1000 - 1/2000. Predicted molecular weight: 38 kDa.
IHC-P 1/100 - 1/500.

Target

  • FunctionHydrolyzes 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.
  • Tissue specificityHighly expressed in liver and kidney, also detected in heart, muscle and brain (at protein level). Not detected in lung.
  • PathwayAmino-acid degradation; L-valine degradation.
  • Involvement in diseaseDefects in HIBCH are the cause of HIBCH deficiency (HIBCHD) [MIM:250620]; 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.
  • Sequence similaritiesBelongs to the enoyl-CoA hydratase/isomerase family.
  • Cellular localizationMitochondrion.
  • Target information above from: UniProt accession Q6NVY1 The UniProt Consortium
    The Universal Protein Resource (UniProt) in 2010
    Nucleic Acids Res. 38:D142-D148 (2010) .

    Information by UniProt
  • Database links
  • Alternative names
    • 3 hydroxyisobutyryl Coenzyme A hydrolase antibody
    • 3 hydroxyisobutyryl Coenzyme A hydrolase, mitochondrial antibody
    • 3-hydroxyisobutyryl-CoA hydrolase antibody
    • 3-hydroxyisobutyryl-coenzyme A hydrolase antibody
    • BETA HYDROXYISOBUTYRYL COENZYME A HYDROLASE antibody
    • HIB CoA hydrolase antibody
    • HIB-CoA hydrolase antibody
    • HIBCH antibody
    • HIBCH_HUMAN antibody
    • HIBCoA hydrolase antibody
    • HIBYL CoA H antibody
    • HIBYL CoAH antibody
    • HIBYL-CoA-H antibody
    • HIBYLCoA H antibody
    • HIBYLCoAH antibody
    • mitochondrial antibody
    see all

Anti-HIBCH antibody images

  • All lanes : Anti-HIBCH antibody (ab101672) at 1/1000 dilution

    Lane 1 : HepG2 lysate
    Lane 2 : Human fetal liver lysate
    Lane 3 : Human fetal kidney lysate
    Lane 4 : Human fetal heart lysate
    Lane 5 : Human fetal brain lysate
    Lane 6 : Human fetal skeletal muscle lysate


    Predicted band size : 38 kDa
  • ab101672 at 1/100 dilution, staining cytoplasmic HIBCH in formalin-fixed, paraffin-embedded Human hepatocarcinoma tissue, by Immunohistochemistry.

References for Anti-HIBCH antibody (ab101672)

ab101672 has not yet been referenced specifically in any publications.

Product Wall


for ab101672:
Antigen retrieval: Sodium citrate buffer (10 mM sodium citrate, pH 6.0), boil for 15 minutes.
Positive control: hepatocarcinoma .


for ab14335:
No antigen retrieval is necessary, the protocol is as follows: Read More

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For ab15563, the protocol states for antigen retrieval "Boiling tissue sections in 10mM citrate buffer, pH 6.0 for 10 min followed by cooling at RT for 20 min. Wash 3 times in buffer."

For ab1215...

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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"