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

SGSH Reaktivität: Ratte WB, IF (cc), IF (p) Wirt: Kaninchen Polyclonal AbBy Fluor® 594
Produktnummer ABIN2806331
  • Target Alle SGSH Antikörper anzeigen
    SGSH (N-Sulfoglucosamine Sulfohydrolase (SGSH))
    Bindungsspezifität
    • 14
    • 13
    • 10
    • 6
    • 4
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    AA 301-388
    Reaktivität
    • 32
    • 18
    • 6
    • 3
    • 3
    • 2
    • 2
    • 1
    • 1
    • 1
    • 1
    Ratte
    Wirt
    • 43
    • 3
    • 1
    Kaninchen
    Klonalität
    • 46
    • 1
    Polyklonal
    Konjugat
    • 23
    • 4
    • 3
    • 3
    • 2
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    Dieser SGSH Antikörper ist konjugiert mit AbBy Fluor® 594
    Applikation
    • 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))
    Kreuzreaktivität
    Ratte
    Homologie
    Human,Mouse,Dog
    Aufreinigung
    Purified by Protein A.
    Immunogen
    KLH conjugated synthetic peptide derived from human Sulphamidase
    Isotyp
    IgG
    Top Product
    Discover our top product SGSH 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
    SGSH (N-Sulfoglucosamine Sulfohydrolase (SGSH))
    Andere Bezeichnung
    SGSH/Sulphamidase (SGSH Produkte)
    Synonyme
    HSS antikoerper, MPS3A antikoerper, SFMD antikoerper, 4632406A19Rik antikoerper, N-sulfoglucosamine sulfohydrolase antikoerper, N-sulfoglucosamine sulfohydrolase (sulfamidase) antikoerper, SGSH antikoerper, Sgsh antikoerper
    Hintergrund

    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.

    Gen-ID
    6448
    UniProt
    P51688
    Pathways
    Glycosaminoglycan Metabolic Process
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