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SGSH 抗体 (AA 301-388)

This anti-SGSH antibody is a 兔 多克隆 antibody detecting SGSH in WB, ELISA, ICC, IF (cc), IF (p), IHC (p) 和 IHC (fro). Suitable for 大鼠.
产品编号 ABIN1386573
发货至: 中国

Quick Overview for SGSH 抗体 (AA 301-388) (ABIN1386573)

抗原

See all SGSH 抗体
SGSH (N-Sulfoglucosamine Sulfohydrolase (SGSH))

适用

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大鼠

宿主

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克隆类型

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多克隆

标记

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This SGSH antibody is un-conjugated

应用范围

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Western Blotting (WB), ELISA, Immunocytochemistry (ICC), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p)), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)), Immunohistochemistry (Frozen Sections) (IHC (fro))
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    AA 301-388

    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
  • 应用备注

    WB 1:300-5000
    ELISA 1:500-1000
    IHC-P 1:200-400
    IHC-F 1:100-500
    IF(IHC-P) 1:50-200
    IF(IHC-F) 1:50-200
    IF(ICC) 1:50-200
    ICC 1:100-500

    限制

    仅限研究用
  • 状态

    Liquid

    浓度

    1 μg/μL

    缓冲液

    0.01M TBS( pH 7.4) with 1 % BSA, 0.02 % 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

    4 °C,-20 °C

    储存方法

    Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.

    有效期

    12 months
  • 抗原

    SGSH (N-Sulfoglucosamine Sulfohydrolase (SGSH))

    别名

    SGSH/Sulphamidase

    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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