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GCSH anticorps (AA 101-173) (Biotin)

GCSH Reactivité: Humain WB, ELISA, IHC (fro), IHC (p) Hôte: Lapin Polyclonal Biotin
N° du produit ABIN1700310
  • Antigène Voir toutes GCSH Anticorps
    GCSH (Glycine Cleavage System H Protein (GCSH))
    Épitope
    • 14
    • 8
    • 7
    • 6
    • 3
    • 2
    AA 101-173
    Reactivité
    • 43
    • 11
    • 11
    • 2
    • 2
    • 2
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    Humain
    Hôte
    • 41
    • 2
    Lapin
    Clonalité
    • 42
    • 1
    Polyclonal
    Conjugué
    • 13
    • 3
    • 3
    • 3
    • 2
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    Cet anticorp GCSH est conjugé à/à la Biotin
    Application
    • 35
    • 18
    • 13
    • 13
    • 3
    • 2
    • 2
    • 2
    • 1
    • 1
    Western Blotting (WB), ELISA, Immunohistochemistry (Frozen Sections) (IHC (fro)), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p))
    Homologie
    Human,Mouse,Rat,Horse
    Purification
    Purified by Protein A.
    Immunogène
    KLH conjugated synthetic peptide derived from human GCSH
    Isotype
    IgG
    Top Product
    Discover our top product GCSH Anticorps primaire
  • Indications d'application
    WB 1:300-5000
    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
    GCSH (Glycine Cleavage System H Protein (GCSH))
    Autre désignation
    GCSH (GCSH Produits)
    Synonymes
    anticorps GCE, anticorps NKH, anticorps GCSH, anticorps H-PROTEIN, anticorps 1100001L02Rik, anticorps 5730591C18Rik, anticorps wu:fb14d10, anticorps wu:fw84b08, anticorps zgc:112535, anticorps BA5228, anticorps gce, anticorps gcsh, anticorps nkh, anticorps wu:fd56h06, anticorps zgc:110557, anticorps zgc:92732, anticorps glycine cleavage system protein H, anticorps glycine cleavage system protein H (aminomethyl carrier), anticorps glycine cleavage system lipoyl carrier protein GcvH, anticorps glycine cleavage system H protein, anticorps glycine cleavage system protein H (aminomethyl carrier), a, anticorps glycine cleavage system carrier of aminomethyl moiety GcvH, anticorps leucine rich repeat containing 34, anticorps glycine cleavage system protein H (aminomethyl carrier) L homeolog, anticorps glycine cleavage system protein H (aminomethyl carrier), b, anticorps GCSH, anticorps Gcsh, anticorps gcvH-2, anticorps gcvH, anticorps gcsha, anticorps LRRC34, anticorps gcsh.L, anticorps gcshb
    Sujet

    Synonyms: GCE, GCSH, GCSH_HUMAN, Glycine cleavage system H protein, Glycine cleavage system H protein mitochondrial, Glycine cleavage system protein H aminomethyl carrier, Glycine cleavage system protein H, Lipoic acid containing protein, mitochondrial, Mitochondrial glycine cleavage system H protein, NKH.

    Background: GCSH is a 173 amino acid mitochondrial protein that contains one lipoyl-binding domain and belongs to the gcvH family. Defects in the gene encoding GCSH are the cause of glycine encephalopathy (GCE), an autosomal recessive disease that is also referred to as non-ketotic hyperglycinemia (NKH). Characterized by severe neurological symptoms, patients with GCE have a large amount of glycine accumulated in their body fluids. The gene encoding GCSH maps to human chromosome 16, which encodes over 900 genes and comprises nearly 3 % of the human genome.

    ID gène
    2653
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