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GCS1 Antikörper (N-Term)

MOGS Reaktivität: Human WB Wirt: Kaninchen Polyclonal RB4961-4962 unconjugated
Produktnummer ABIN389016
  • Target Alle GCS1 (MOGS) Antikörper anzeigen
    GCS1 (MOGS) (Mannosyl-Oligosaccharide Glucosidase (MOGS))
    Bindungsspezifität
    • 15
    • 7
    • 6
    • 4
    • 3
    • 1
    • 1
    AA 1-30, N-Term
    Reaktivität
    • 40
    • 14
    • 4
    • 2
    • 2
    • 2
    • 2
    • 2
    • 2
    • 1
    • 1
    • 1
    Human
    Wirt
    • 41
    Kaninchen
    Klonalität
    • 41
    Polyklonal
    Konjugat
    • 17
    • 3
    • 3
    • 3
    • 2
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    Dieser GCS1 Antikörper ist unkonjugiert
    Applikation
    • 41
    • 18
    • 13
    • 13
    • 3
    • 3
    • 3
    • 2
    • 2
    • 2
    • 1
    Western Blotting (WB)
    Aufreinigung
    This antibody is prepared by Saturated Ammonium Sulfate (SAS) precipitation followed by dialysis against PBS.
    Immunogen
    This GCS1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 1-30 amino acids from the N-terminal region of human GCS1.
    Klon
    RB4961-4962
    Isotyp
    Ig Fraction
  • Applikationshinweise
    WB: 1:1000
    Beschränkungen
    Nur für Forschungszwecke einsetzbar
  • Format
    Liquid
    Buffer
    Purified polyclonal antibody supplied in PBS with 0.09 % (W/V) sodium azide.
    Konservierungsmittel
    Sodium azide
    Vorsichtsmaßnahmen
    This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
    Lagerung
    4 °C,-20 °C
    Informationen zur Lagerung
    Maintain refrigerated at 2-8 °C for up to 6 months. For long term storage store at -20 °C in small aliquots to prevent freeze-thaw cycles.
    Haltbarkeit
    6 months
  • Target
    GCS1 (MOGS) (Mannosyl-Oligosaccharide Glucosidase (MOGS))
    Andere Bezeichnung
    GCS1 (MOGS Produkte)
    Synonyme
    Afu6g04210 antikoerper, AO090701000141 antikoerper, Mogs antikoerper, CDG2B antikoerper, CWH41 antikoerper, DER7 antikoerper, GCS1 antikoerper, 1810017N02Rik antikoerper, AI181835 antikoerper, Gcs1 antikoerper, gcs1 antikoerper, im:7160827 antikoerper, wu:fe50a12 antikoerper, wu:fk09a10 antikoerper, zgc:158312 antikoerper, mannosyl-oligosaccharide glucosidase antikoerper, mannosyl-oligosaccharide glucosidase GCS1 antikoerper, mannosyl-oligosaccharide glucosidase L homeolog antikoerper, mannosyl oligosaccharide glucosidase antikoerper, glucosidase 1 antikoerper, AFUA_6G04210 antikoerper, Tc00.1047053511015.10 antikoerper, Tc00.1047053511805.10 antikoerper, LOC5576381 antikoerper, AOR_1_260114 antikoerper, MGYG_00305 antikoerper, TERG_01248 antikoerper, mogs.L antikoerper, TTHERM_00636930 antikoerper, LOAG_03690 antikoerper, Gcs1 antikoerper, MOGS antikoerper, Mogs antikoerper, mogs antikoerper
    Hintergrund
    GCS1 cleaves the distal alpha 1,2-linked glucose residue from the Glc(3)Man(9)GlcNAc(2) oligosaccharide precursor in a highly specific manner. Defects in GCS1 are the cause of type IIb congenital disorder of glycosylation (CDGIIb). This syndrome is also known as glucosidase I deficiency and is characterized by marked generalized hypotonia and hypomotility of the neonate, dysmorphic features, including a prominent occiput, short palpebral fissures, retrognathia, high arched palate, generalized edema, and hypoplastic genitalia. Symptoms include hepatomegaly, hypoventilation, feeding problems and seizures. The clinical course is progressive and survival is at most a few months.
    Molekulargewicht
    91918
    Gen-ID
    7841
    NCBI Accession
    NP_001139630, NP_006293
    UniProt
    Q13724
    Pathways
    SARS-CoV-2 Protein Interaktom
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