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GCSH Antikörper (AA 101-173) (Cy3)

GCSH Reaktivität: Human WB, IF (cc), IF (p) Wirt: Kaninchen Polyclonal Cy3
Produktnummer ABIN1702123
  • Target Alle GCSH Antikörper anzeigen
    GCSH (Glycine Cleavage System H Protein (GCSH))
    Bindungsspezifität
    • 14
    • 8
    • 7
    • 6
    • 3
    • 2
    AA 101-173
    Reaktivität
    • 43
    • 11
    • 11
    • 2
    • 2
    • 2
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    Human
    Wirt
    • 41
    • 2
    Kaninchen
    Klonalität
    • 42
    • 1
    Polyklonal
    Konjugat
    • 13
    • 4
    • 3
    • 3
    • 2
    • 2
    • 2
    • 2
    • 2
    • 2
    • 2
    • 2
    • 1
    • 1
    • 1
    • 1
    Dieser GCSH Antikörper ist konjugiert mit Cy3
    Applikation
    • 35
    • 19
    • 12
    • 12
    • 3
    • 3
    • 3
    • 2
    • 1
    • 1
    Western Blotting (WB), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))
    Homologie
    Human,Mouse,Rat,Horse
    Aufreinigung
    Purified by Protein A.
    Immunogen
    KLH conjugated synthetic peptide derived from human GCSH
    Isotyp
    IgG
    Top Product
    Discover our top product GCSH Primärantikörper
  • Applikationshinweise
    IF(IHC-P) 1:50-200
    IF(IHC-F) 1:50-200
    IF(ICC) 1:50-200
    Beschränkungen
    Nur für Forschungszwecke einsetzbar
  • Format
    Liquid
    Konzentration
    1 μg/μL
    Buffer
    Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.
    Konservierungsmittel
    ProClin
    Vorsichtsmaßnahmen
    This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.
    Lagerung
    -20 °C
    Informationen zur Lagerung
    Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
    Haltbarkeit
    12 months
  • Target
    GCSH (Glycine Cleavage System H Protein (GCSH))
    Andere Bezeichnung
    GCSH (GCSH Produkte)
    Synonyme
    GCE antikoerper, NKH antikoerper, GCSH antikoerper, H-PROTEIN antikoerper, 1100001L02Rik antikoerper, 5730591C18Rik antikoerper, wu:fb14d10 antikoerper, wu:fw84b08 antikoerper, zgc:112535 antikoerper, BA5228 antikoerper, gce antikoerper, gcsh antikoerper, nkh antikoerper, wu:fd56h06 antikoerper, zgc:110557 antikoerper, zgc:92732 antikoerper, glycine cleavage system protein H antikoerper, glycine cleavage system protein H (aminomethyl carrier) antikoerper, glycine cleavage system lipoyl carrier protein GcvH antikoerper, glycine cleavage system H protein antikoerper, glycine cleavage system protein H (aminomethyl carrier), a antikoerper, glycine cleavage system carrier of aminomethyl moiety GcvH antikoerper, leucine rich repeat containing 34 antikoerper, glycine cleavage system protein H (aminomethyl carrier) L homeolog antikoerper, glycine cleavage system protein H (aminomethyl carrier), b antikoerper, GCSH antikoerper, Gcsh antikoerper, gcvH-2 antikoerper, gcvH antikoerper, gcsha antikoerper, LRRC34 antikoerper, gcsh.L antikoerper, gcshb antikoerper
    Hintergrund

    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.

    Gen-ID
    2653
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