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SGSH anticorps (AA 301-388) (AbBy Fluor® 594)

SGSH Reactivité: Rat WB, IF (cc), IF (p) Hôte: Lapin Polyclonal AbBy Fluor® 594
N° du produit ABIN2806331
  • Antigène Voir toutes SGSH Anticorps
    SGSH (N-Sulfoglucosamine Sulfohydrolase (SGSH))
    Épitope
    • 14
    • 13
    • 10
    • 6
    • 4
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    AA 301-388
    Reactivité
    • 32
    • 18
    • 6
    • 3
    • 3
    • 2
    • 2
    • 1
    • 1
    • 1
    • 1
    Rat
    Hôte
    • 43
    • 3
    • 1
    Lapin
    Clonalité
    • 46
    • 1
    Polyclonal
    Conjugué
    • 23
    • 4
    • 3
    • 3
    • 2
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    Cet anticorp SGSH est conjugé à/à la AbBy Fluor® 594
    Application
    • 45
    • 17
    • 12
    • 12
    • 7
    • 4
    • 4
    • 3
    • 2
    • 2
    • 1
    • 1
    • 1
    Western Blotting (WB), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))
     Réactivité croisée
    Rat
    Homologie
    Human,Mouse,Dog
    Purification
    Purified by Protein A.
    Immunogène
    KLH conjugated synthetic peptide derived from human Sulphamidase
    Isotype
    IgG
    Top Product
    Discover our top product SGSH Anticorps primaire
  • Indications d'application
    IF(IHC-P) 1:50-200
    IF(IHC-F) 1:50-200
    IF(ICC) 1:50-200
    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. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
    Date de péremption
    12 months
  • Antigène
    SGSH (N-Sulfoglucosamine Sulfohydrolase (SGSH))
    Autre désignation
    SGSH/Sulphamidase (SGSH Produits)
    Synonymes
    anticorps HSS, anticorps MPS3A, anticorps SFMD, anticorps 4632406A19Rik, anticorps N-sulfoglucosamine sulfohydrolase, anticorps N-sulfoglucosamine sulfohydrolase (sulfamidase), anticorps SGSH, anticorps Sgsh
    Sujet

    Synonyms: HSS, SFMD, MPS3A, N-sulphoglucosamine sulphohydrolase, Sulfoglucosamine sulfamidase, Sulphamidase, SGSH

    Background: Sulfatases are enzymes that hydrolyse a diverse range of sulfate esters. Deficiency of lysosomal sulfatases leads to human diseases characterized by the accumulation of either GAGs (glycosaminoglycans) or sulfolipids. Sulfamidase, also known as HSS, SFMD, MPS3A or SGSH, is a 502 amino acid lysosome that belongs to the sulfatase family. It has been suggested that sulfamidase may be involved in the lysosomal degradation of heparan sulfate. Defects in the gene encoding sulfamidase are the cause of Sanfilippo syndrome A, an autosomal recessive lysosomal storage disease caused by impaired degradation of heparan sulfate. Sanfilippo syndrome A is characterized by severe central nervous system degeneration but relatively mild somatic manifestations.

    ID gène
    6448
    UniProt
    P51688
    Pathways
    Glycosaminoglycan Metabolic Process
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