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ETHE1 anticorps (AA 191-254) (Biotin)

ETHE1 Reactivité: Humain ELISA, IHC (p), IHC (fro) Hôte: Lapin Polyclonal Biotin
N° du produit ABIN895785
  • Antigène Voir toutes ETHE1 Anticorps
    ETHE1 (Ethylmalonic Encephalopathy 1 (ETHE1))
    Épitope
    • 14
    • 6
    • 3
    • 2
    • 2
    • 2
    • 1
    • 1
    • 1
    AA 191-254
    Reactivité
    • 41
    • 18
    • 3
    • 2
    • 2
    • 2
    • 2
    Humain
    Hôte
    • 39
    • 2
    Lapin
    Clonalité
    • 41
    Polyclonal
    Conjugué
    • 17
    • 3
    • 2
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    Cet anticorp ETHE1 est conjugé à/à la Biotin
    Application
    • 16
    • 13
    • 13
    • 9
    • 7
    • 7
    • 4
    • 2
    • 2
    • 2
    ELISA, Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)), Immunohistochemistry (Frozen Sections) (IHC (fro))
    Homologie
    Human,Mouse,Dog,Cow,Sheep,Pig,Rabbit
    Purification
    Purified by Protein A.
    Immunogène
    KLH conjugated synthetic peptide derived from human E1
    Isotype
    IgG
    Top Product
    Discover our top product ETHE1 Anticorps primaire
  • Indications d'application
    IHC-P 1:200-400
    IHC-F 1:100-500
    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 for 12 months.
    Date de péremption
    12 months
  • Antigène
    ETHE1 (Ethylmalonic Encephalopathy 1 (ETHE1))
    Autre désignation
    ETHE1 (ETHE1 Produits)
    Synonymes
    anticorps zgc:85680, anticorps HSCO, anticorps YF13H12, anticorps 0610025L15Rik, anticorps Hsco, anticorps ETHE1, persulfide dioxygenase, anticorps ethylmalonic encephalopathy 1, anticorps Ethe1, anticorps ethe1, anticorps ETHE1
    Sujet

    Synonyms: mitochondrial, Ethe1, ETHE1 protein, mitochondrial precursor, ETHE1_HUMAN, ethylmalonic encephalopathy 1, Ethylmalonic encephalopathy protein 1, hepatoma subtracted clone one, Hepatoma subtracted clone one protein, HSCO, Protein ETHE1, YF13H12.

    Background: Probably plays an important role in metabolic homeostasis in mitochondria. May function as a nuclear-cytoplasmic shuttling protein that binds transcription factor RELA/NFKB3 in the nucleus and exports it to the cytoplasm. Suppresses p53-induced apoptosis by preventing nuclear localization of RELA.Involvement in disease:Defects in ETHE1 are a cause of ethylmalonic encephalopathy (EE) . EE is an autosomal recessive disorder characterized by neurodevelopmental delay and regression, recurrent petechiae, acrocyanosis, diarrhea, leading to death in the first decade of life. It is also associated with persistent lactic acidemia and ethylmalonic and methylsuccinic aciduria.

    ID gène
    23474
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