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GCS1 anticorps (N-Term)

MOGS Reactivité: Humain WB Hôte: Lapin Polyclonal RB4961-4962 unconjugated
N° du produit ABIN389016
  • Antigène Voir toutes GCS1 (MOGS) Anticorps
    GCS1 (MOGS) (Mannosyl-Oligosaccharide Glucosidase (MOGS))
    Épitope
    • 15
    • 9
    • 9
    • 7
    • 6
    • 1
    • 1
    AA 1-30, N-Term
    Reactivité
    • 52
    • 14
    • 4
    • 2
    • 2
    • 2
    • 2
    • 2
    • 2
    • 1
    • 1
    • 1
    Humain
    Hôte
    • 53
    Lapin
    Clonalité
    • 53
    Polyclonal
    Conjugué
    • 19
    • 5
    • 5
    • 5
    • 4
    • 4
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    Cet anticorp GCS1 est non-conjugé
    Application
    • 53
    • 31
    • 13
    • 13
    • 3
    • 3
    • 3
    • 2
    • 2
    • 2
    • 1
    Western Blotting (WB)
    Purification
    This antibody is prepared by Saturated Ammonium Sulfate (SAS) precipitation followed by dialysis against PBS.
    Immunogène
    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.
    Clone
    RB4961-4962
    Isotype
    Ig Fraction
    Top Product
    Discover our top product MOGS Anticorps primaire
  • Indications d'application
    WB: 1:1000
    Restrictions
    For Research Use only
  • Format
    Liquid
    Buffer
    Purified polyclonal antibody supplied in PBS with 0.09 % (W/V) sodium azide.
    Agent conservateur
    Sodium azide
    Précaution d'utilisation
    This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
    Stock
    4 °C,-20 °C
    Stockage commentaire
    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.
    Date de péremption
    6 months
  • Antigène
    GCS1 (MOGS) (Mannosyl-Oligosaccharide Glucosidase (MOGS))
    Autre désignation
    GCS1 (MOGS Produits)
    Synonymes
    anticorps Afu6g04210, anticorps AO090701000141, anticorps Mogs, anticorps CDG2B, anticorps CWH41, anticorps DER7, anticorps GCS1, anticorps 1810017N02Rik, anticorps AI181835, anticorps Gcs1, anticorps gcs1, anticorps im:7160827, anticorps wu:fe50a12, anticorps wu:fk09a10, anticorps zgc:158312, anticorps mannosyl-oligosaccharide glucosidase, anticorps mannosyl-oligosaccharide glucosidase GCS1, anticorps mannosyl-oligosaccharide glucosidase L homeolog, anticorps mannosyl oligosaccharide glucosidase, anticorps glucosidase 1, anticorps AFUA_6G04210, anticorps Tc00.1047053511015.10, anticorps Tc00.1047053511805.10, anticorps LOC5576381, anticorps AOR_1_260114, anticorps MGYG_00305, anticorps TERG_01248, anticorps mogs.L, anticorps TTHERM_00636930, anticorps LOAG_03690, anticorps Gcs1, anticorps MOGS, anticorps Mogs, anticorps mogs
    Sujet
    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.
    Poids moléculaire
    91918
    ID gène
    7841
    NCBI Accession
    NP_001139630, NP_006293
    UniProt
    Q13724
    Pathways
    SARS-CoV-2 Protein Interactome
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