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

HIBCH Reactivité: Humain WB, IF (cc), IF (p) Hôte: Lapin Polyclonal AbBy Fluor® 750
N° du produit ABIN5004616
  • Antigène Voir toutes HIBCH Anticorps
    HIBCH (3-hydroxyisobutyryl-CoA Hydrolase (HIBCH))
    Épitope
    • 14
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    AA 251-350
    Reactivité
    • 29
    • 6
    • 5
    • 3
    • 2
    Humain
    Hôte
    • 22
    • 8
    Lapin
    Clonalité
    • 22
    • 8
    Polyclonal
    Conjugué
    • 17
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    Cet anticorp HIBCH est conjugé à/à la 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))
    Homologie
    Human
    Purification
    Purified by Protein A.
    Immunogène
    KLH conjugated synthetic peptide derived from human HIBCH
    Isotype
    IgG
    Top Product
    Discover our top product HIBCH Anticorps primaire
  • Indications d'application
    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.
    Agent conservateur
    ProClin
    Précaution d'utilisation
    This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.
    Stock
    -20 °C
    Stockage commentaire
    Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
    Date de péremption
    12 months
  • Antigène
    HIBCH (3-hydroxyisobutyryl-CoA Hydrolase (HIBCH))
    Autre désignation
    HIBCH (HIBCH Produits)
    Synonymes
    anticorps DDBDRAFT_0189353, anticorps DDBDRAFT_0233831, anticorps DDB_0189353, anticorps DDB_0233831, anticorps T11J7.4, anticorps T11J7_4, anticorps T11J7.5, anticorps T11J7_5, anticorps HIBYLCOAH, anticorps 2610509I15Rik, anticorps AI648812, anticorps zgc:110824, anticorps 3-hydroxyisobutyryl-coenzyme A hydrolase, anticorps 3-hydroxyisobutyryl-coenzyme a hydrolase,putative, anticorps 3-hydroxyisobutyryl-Coenzyme A hydrolase, anticorps 3-hydroxyisobutyryl-CoA hydrolase (predicted), anticorps 3-hydroxyisobutyryl-CoA hydrolase, anticorps ATP-dependent caseinolytic (Clp) protease/crotonase family protein, anticorps putative 3-hydroxyisobutyryl-coenzyme A hydrolase, anticorps 3-hydroxyisobutyryl-CoA hydrolase L homeolog, anticorps PVX_100950, anticorps CpipJ_CPIJ012030, anticorps PKH_145800, anticorps hibch, anticorps MCYG_05070, anticorps SPBC2D10.09, anticorps CNA02940, anticorps LACBIDRAFT_292320, anticorps SJAG_00944, anticorps VDBG_07520, anticorps AT2G30650, anticorps AT2G30660, anticorps LBRM_32_3920, anticorps HIBCH, anticorps Hibch, anticorps hibch.L
    Sujet

    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.

    ID gène
    26275
    Pathways
    Monocarboxylic Acid Catabolic Process
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