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HIBADH anticorps (AA 251-336) (AbBy Fluor® 488)

HIBADH Reactivité: Humain, Souris WB, IF (cc), IF (p) Hôte: Lapin Polyclonal AbBy Fluor® 488
N° du produit ABIN1694580
  • Antigène Voir toutes HIBADH Anticorps
    HIBADH (3-hydroxyisobutyrate Dehydrogenase (HIBADH))
    Épitope
    • 14
    • 14
    • 7
    • 4
    • 4
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    AA 251-336
    Reactivité
    • 51
    • 33
    • 11
    • 5
    • 4
    • 4
    • 4
    • 3
    • 2
    • 2
    • 2
    • 2
    • 2
    • 1
    Humain, Souris
    Hôte
    • 49
    • 2
    Lapin
    Clonalité
    • 51
    Polyclonal
    Conjugué
    • 22
    • 3
    • 3
    • 3
    • 2
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    Cet anticorp HIBADH est conjugé à/à la AbBy Fluor® 488
    Application
    • 41
    • 20
    • 12
    • 12
    • 7
    • 7
    • 3
    • 3
    • 2
    Western Blotting (WB), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))
     Réactivité croisée
    Humain, Souris
    Homologie
    Rat,Cow,Sheep,Horse
    Purification
    Purified by Protein A.
    Immunogène
    KLH conjugated synthetic peptide derived from human HIBADH
    Isotype
    IgG
    Top Product
    Discover our top product HIBADH 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
    HIBADH (3-hydroxyisobutyrate Dehydrogenase (HIBADH))
    Autre désignation
    Hibadh (HIBADH Produits)
    Synonymes
    anticorps NS5ATP1, anticorps 6430402H10Rik, anticorps AI265272, anticorps hibadh, anticorps zgc:66262, anticorps wu:fb07f02, anticorps zgc:100804, anticorps 3-hydroxyisobutyrate dehydrogenase, anticorps 3-hydroxyisobutyrate dehydrogenase b, anticorps 3-hydroxyisobutyrate dehydrogenase S homeolog, anticorps 3-hydroxyisobutyrate dehydrogenase a, anticorps HIBADH, anticorps Hibadh, anticorps hibadhb, anticorps hibadh.S, anticorps hibadha
    Sujet

    Synonyms: 3 hydroxy 2 methylpropanoate:NAD+ oxidoreductase, 3 hydroxyisobutyrate dehydrogenase, 3 hydroxyisobutyrate dehydrogenase mitochondrial, EC 1.1.1.31, MGC40361, NS5ATP1, 3HIDH_HUMAN.

    Background: HIBADH is a 336 amino acid mitochondrial enzyme that catalyzes the NAD+-dependent, reversible oxidization of 3-Hydroxyisobutyrate to methylmalonate semialdehyde, an intermediate of valine catabolism. The enzyme functions as a homodimer between a pH of 7.0 and 10.0, with optimal activity between 8.8 and 9.0. It was previously hypothesized that defects in the gene encoding HIBADH may be the cause of 3-Hydroxyisobutyric aciduria, a rare disorder that is characterized by a variety of clinical manifestations such as neurodevelopmental problems and dysmorphic features. However, it was shown that HIBADH activity was equal in patients with 3-Hydroxyisobutyric aciduria as compared with controls.

    ID gène
    11112
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