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GNS 抗体 (AA 1-100) (Biotin)

This Biotin-conjugated 兔 多克隆 anti-GNS antibody specifically detects GNS in ELISA, IHC (p) 和 IHC (fro). The antibody is reactive with 人 samples.
产品编号 ABIN1700879
发货至: 中国
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中国
北京 101111
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Beijing Economic Technological Development Area
Room 801-803
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Tel +86 (0512) 65829739 传真 +86 (010) 6788 5057

Quick Overview for GNS 抗体 (AA 1-100) (Biotin) (ABIN1700879)

抗原

See all GNS 抗体
GNS (Glucosamine (N-Acetyl)-6-Sulfatase (GNS))

适用

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宿主

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克隆类型

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多克隆

标记

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This GNS antibody is conjugated to Biotin

应用范围

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ELISA, Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)), Immunohistochemistry (Frozen Sections) (IHC (fro))
  • 抗原表位

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    AA 1-100

    预测反应

    Human,Mouse,Rat,Cow,Horse,Rabbit

    纯化方法

    Purified by Protein A.

    免疫原

    KLH conjugated synthetic peptide derived from human GNS/Glucosamine 6 sulfatase

    亚型

    IgG
  • 应用备注

    IHC-P 1:200-400
    IHC-F 1:100-500

    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 for 12 months.

    有效期

    12 months
  • 抗原

    GNS (Glucosamine (N-Acetyl)-6-Sulfatase (GNS))

    别名

    Glucosamine 6 sulfatase

    背景

    Synonyms: 2610016K11Rik, AU042285, C87209, G6S, Glucosamine N-acetyl 6 sulfatase, Glucosamine 6 sulfatase, Glucosamine-6-sulfatase, GNS, GNS_HUMAN, MGC21274, N acetylglucosamine 6 sulfatase [Precursor], N-acetylglucosamine-6-sulfatase, N28088.

    Background: GNS is a 552 amino acid lysosomal enzyme that hydrolyzes the 6-sulfate groups of the N-acetyl-D-glucosamine 6-sulfate units of keratan sulfate and heparan sulfate. A member of the sulfatase family, GNS assists in the catabolism of heparin, and binds calcium as a cofactor. GNS deficiency results in an autosomal recessive lysosomal storage disorder known as mucopolysaccharidosis type IIID (Sanfilippo D syndrome), which is characterized by mild somatic disease and severe degeneration of the central nervous system. Subject to post-translational internal peptidase cleavage, GNS is encoded by a gene mapping to human chromosome 12q14.2 and mouse chromosome 10 D2.

    基因ID

    2799

    途径

    Glycosaminoglycan Metabolic Process
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