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HIBCH antibody (AA 251-350) (AbBy Fluor® 750)

HIBCH Reactivity: Human WB, IF (cc), IF (p) Host: Rabbit Polyclonal AbBy Fluor® 750
Catalog No. ABIN5004616
  • Target See all HIBCH Antibodies
    HIBCH (3-hydroxyisobutyryl-CoA Hydrolase (HIBCH))
    Binding Specificity
    • 14
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    AA 251-350
    Reactivity
    • 29
    • 6
    • 5
    • 3
    • 2
    Human
    Host
    • 22
    • 8
    Rabbit
    Clonality
    • 22
    • 8
    Polyclonal
    Conjugate
    • 17
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    This HIBCH antibody is conjugated to AbBy Fluor® 750
    Application
    • 29
    • 12
    • 12
    • 9
    • 8
    • 6
    • 5
    • 4
    • 4
    • 3
    • 1
    • 1
    Western Blotting (WB), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))
    Predicted Reactivity
    Human
    Purification
    Purified by Protein A.
    Immunogen
    KLH conjugated synthetic peptide derived from human HIBCH
    Isotype
    IgG
    Top Product
    Discover our top product HIBCH Primary Antibody
  • Application Notes
    IF(IHC-P) 1:50-200
    IF(IHC-F) 1:50-200
    IF(ICC) 1:50-200
    Restrictions
    For Research Use only
  • Format
    Liquid
    Concentration
    1 μg/μL
    Buffer
    Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.
    Preservative
    ProClin
    Precaution of Use
    This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.
    Storage
    -20 °C
    Storage Comment
    Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
    Expiry Date
    12 months
  • Target
    HIBCH (3-hydroxyisobutyryl-CoA Hydrolase (HIBCH))
    Alternative Name
    HIBCH (HIBCH Products)
    Synonyms
    DDBDRAFT_0189353 antibody, DDBDRAFT_0233831 antibody, DDB_0189353 antibody, DDB_0233831 antibody, T11J7.4 antibody, T11J7_4 antibody, T11J7.5 antibody, T11J7_5 antibody, HIBYLCOAH antibody, 2610509I15Rik antibody, AI648812 antibody, zgc:110824 antibody, 3-hydroxyisobutyryl-coenzyme A hydrolase antibody, 3-hydroxyisobutyryl-coenzyme a hydrolase,putative antibody, 3-hydroxyisobutyryl-Coenzyme A hydrolase antibody, 3-hydroxyisobutyryl-CoA hydrolase (predicted) antibody, 3-hydroxyisobutyryl-CoA hydrolase antibody, ATP-dependent caseinolytic (Clp) protease/crotonase family protein antibody, putative 3-hydroxyisobutyryl-coenzyme A hydrolase antibody, 3-hydroxyisobutyryl-CoA hydrolase L homeolog antibody, PVX_100950 antibody, CpipJ_CPIJ012030 antibody, PKH_145800 antibody, hibch antibody, MCYG_05070 antibody, SPBC2D10.09 antibody, CNA02940 antibody, LACBIDRAFT_292320 antibody, SJAG_00944 antibody, VDBG_07520 antibody, AT2G30650 antibody, AT2G30660 antibody, LBRM_32_3920 antibody, HIBCH antibody, Hibch antibody, hibch.L antibody
    Background

    Synonyms: 3 hydroxyisobutyryl Coenzyme A hydrolase, 3 hydroxyisobutyryl Coenzyme A hydrolase, mitochondrial, 3-hydroxyisobutyryl-CoA hydrolase, 3-hydroxyisobutyryl-coenzyme A hydrolase, BETA HYDROXYISOBUTYRYL COENZYME A HYDROLASE, HIB CoA hydrolase, HIB-CoA hydrolase, HIBCH, HIBCH_HUMAN, HIBCoA hydrolase, HIBYL CoA H, HIBYL CoAH, HIBYL-CoA-H, HIBYLCoA H, HIBYLCoAH, mitochondrial, HIBCH_HUMAN.

    Background: 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 _-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 _-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.

    Gene ID
    26275
    Pathways
    Monocarboxylic Acid Catabolic Process
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