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SGSH 抗体 (AA 301-388) (AbBy Fluor® 647)

SGSH 适用: 大鼠 WB, IF (cc), IF (p) 宿主: 兔 Polyclonal AbBy Fluor® 647
产品编号 ABIN1393140
发货至: 中国
  • 抗原 See all SGSH 抗体
    SGSH (N-Sulfoglucosamine Sulfohydrolase (SGSH))
    抗原表位
    • 14
    • 13
    • 10
    • 6
    • 4
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    AA 301-388
    适用
    • 32
    • 18
    • 6
    • 3
    • 3
    • 2
    • 2
    • 1
    • 1
    • 1
    • 1
    大鼠
    宿主
    • 43
    • 3
    • 1
    克隆类型
    • 46
    • 1
    多克隆
    标记
    • 23
    • 4
    • 3
    • 3
    • 2
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    This SGSH antibody is conjugated to AbBy Fluor® 647
    应用范围
    • 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))
    交叉反应
    大鼠
    预测反应
    Human,Mouse,Dog
    纯化方法
    Purified by Protein A.
    免疫原
    KLH conjugated synthetic peptide derived from human Sulphamidase
    亚型
    IgG
    Top Product
    Discover our top product SGSH Primary Antibody
  • 应用备注
    IF(IHC-P) 1:50-200
    IF(IHC-F) 1:50-200
    IF(ICC) 1:50-200
    限制
    仅限研究用
  • 状态
    Liquid
    浓度
    1 μg/μL
    缓冲液
    Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.
    储存液
    ProClin
    注意事项
    This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.
    储存条件
    -20 °C
    储存方法
    Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
    有效期
    12 months
  • 抗原
    SGSH (N-Sulfoglucosamine Sulfohydrolase (SGSH))
    别名
    SGSH/Sulphamidase (SGSH 产品)
    别名
    HSS antibody, MPS3A antibody, SFMD antibody, 4632406A19Rik antibody, N-sulfoglucosamine sulfohydrolase antibody, N-sulfoglucosamine sulfohydrolase (sulfamidase) antibody, SGSH antibody, Sgsh antibody
    背景

    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.

    基因ID
    6448
    UniProt
    P51688
    途径
    Glycosaminoglycan Metabolic Process
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