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SGSH antibody (AA 301-388) (AbBy Fluor® 680)

SGSH Reactivity: Rat WB, IF (cc), IF (p) Host: Rabbit Polyclonal AbBy Fluor® 680
Catalog No. ABIN5010456
  • Target See all SGSH Antibodies
    SGSH (N-Sulfoglucosamine Sulfohydrolase (SGSH))
    Binding Specificity
    • 14
    • 13
    • 10
    • 6
    • 4
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    AA 301-388
    Reactivity
    • 32
    • 18
    • 6
    • 3
    • 3
    • 2
    • 2
    • 1
    • 1
    • 1
    • 1
    Rat
    Host
    • 43
    • 3
    • 1
    Rabbit
    Clonality
    • 46
    • 1
    Polyclonal
    Conjugate
    • 23
    • 4
    • 3
    • 3
    • 2
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    This SGSH antibody is conjugated to AbBy Fluor® 680
    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))
    Cross-Reactivity
    Rat
    Predicted Reactivity
    Human,Mouse,Dog
    Purification
    Purified by Protein A.
    Immunogen
    KLH conjugated synthetic peptide derived from human Sulphamidase
    Isotype
    IgG
    Top Product
    Discover our top product SGSH Primary Antibody
  • Application Notes
    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.
    Preservative
    ProClin
    Precaution of Use
    This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.
    Storage
    -20 °C
    Storage Comment
    Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
    Expiry Date
    12 months
  • Target
    SGSH (N-Sulfoglucosamine Sulfohydrolase (SGSH))
    Alternative Name
    SGSH/Sulphamidase (SGSH Products)
    Synonyms
    HSS antibody, MPS3A antibody, SFMD antibody, 4632406A19Rik antibody, N-sulfoglucosamine sulfohydrolase antibody, N-sulfoglucosamine sulfohydrolase (sulfamidase) antibody, SGSH antibody, Sgsh antibody
    Background

    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.

    Gene ID
    6448
    UniProt
    P51688
    Pathways
    Glycosaminoglycan Metabolic Process
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