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    SGSH/Sulphamidase Polyclonal Antibody, ALEXA FLUOR® 555 Conjugated

    Overview
    Catalog # bs-11756R-A555
    Product Name SGSH/Sulphamidase Polyclonal Antibody, ALEXA FLUOR® 555 Conjugated
    Applications IF(IHC-P)
    Reactivity Human, Mouse, Rat
    Specifications
    Conjugation ALEXA FLUOR® 555
    Host Rabbit
    Source KLH conjugated synthetic peptide derived from human Sulphamidase
    Immunogen Range 310-360/502
    Clonality Polyclonal
    Isotype IgG
    Concentration 1ug/ul
    Purification Purified by Protein A.
    Storage Aqueous buffered solution containing 1% BSA, 50% glycerol and 0.09% sodium azide. Store at 4°C for 12 months.
    Target
    Gene ID 6448
    Swiss Prot P51688
    Subcellular location Lysosome
    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.