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Merck
CN

HPA030200

Sigma-Aldrich

抗-DST 兔抗

Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution

别名:

Anti-BP240, Anti-BPA, Anti-BPAG1, Anti-CATX-15, Anti-FLJ13425, Anti-FLJ21489, Anti-FLJ30627, Anti-FLJ32235, Anti-KIAA0728, Anti-MACF2, Anti-dystonin

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关于此项目

UNSPSC代码:
12352203
人类蛋白质图谱编号:
NACRES:
NA.41
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生物来源

rabbit

质量水平

偶联物

unconjugated

抗体形式

affinity isolated antibody

抗体产品类型

primary antibodies

克隆

polyclonal

产品线

Prestige Antibodies® Powered by Atlas Antibodies

表单

buffered aqueous glycerol solution

种属反应性

human

技术

immunofluorescence: 0.25-2 μg/mL
immunohistochemistry: 1:50-1:200

免疫原序列

KPRCQNVAEDMDIQLCASILNENSDENENINTMILLDKMHSCSSLEKQQRVNVVQLASPSENNLVTEKSNLPEYTTEIAGKSKENLLNHE

UniProt登记号

运输

wet ice

储存温度

−20°C

靶向翻译后修饰

unmodified

基因信息

human ... DST(667)

一般描述

DST gene encodes dystonin, a cytoskeletal linker protein. Dystonin proteins are present as several isoforms in neural and muscle cells. DST gene is located on the human chromosome 6p12.1. Dystonin protein encompasses plakin and spectrin domains.

免疫原

dystonin recombinant protein epitope signature tag (PrEST)

应用

Anti-DST antibody produced in rabbit is suitable for human dystonin detection in live cell microscopy.

生化/生理作用

DST integrates microtubules, actin and intermediate filaments and is essential for cytoskeleton network in neuronal cells. A frame shift mutation in the DST gene affects sensory and autonomous nervous system, leading to hereditary sensory autonomic neuropathy type VI. The incidence of nonsense mutation in the DST gene is implicated in the skin disorder.

特点和优势

Prestige Antibodies® are highly characterized and extensively validated antibodies with the added benefit of all available characterization data for each target being accessible via the Human Protein Atlas portal linked just below the product name at the top of this page. The uniqueness and low cross-reactivity of the Prestige Antibodies® to other proteins are due to a thorough selection of antigen regions, affinity purification, and stringent selection. Prestige antigen controls are available for every corresponding Prestige Antibody and can be found in the linkage section.

Every Prestige Antibody is tested in the following ways:
  • IHC tissue array of 44 normal human tissues and 20 of the most common cancer type tissues.
  • Protein array of 364 human recombinant protein fragments.

外形

Solution in phosphate-buffered saline, pH 7.2, containing 40% glycerol and 0.02% sodium azide.

其他说明

Corresponding Antigen APREST77625

法律信息

Prestige Antibodies is a registered trademark of Merck KGaA, Darmstadt, Germany

免责声明

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

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储存分类代码

10 - Combustible liquids

WGK

WGK 1

闪点(°F)

Not applicable

闪点(°C)

Not applicable

法规信息

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历史批次信息供参考:

分析证书(COA)

Lot/Batch Number

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访问文档库

Hereditary sensory autonomic neuropathy caused by a mutation in dystonin
Edvardson S, et al.
Annals of Neurology, 71(4), 569-572 (2012)
Novel mutations in dystonin provide clues to the pathomechanisms of HSAN-VI
Manganelli F, et al.
Neurology, 88(22), 2132-2140 (2017)
Cloning and characterization of the neural isoforms of human dystonin
Brown A, et al.
Genomics, 29(3), 777-780 (1995)
A homozygous nonsense mutation within the dystonin gene coding for the coiled-coil domain of the epithelial isoform of BPAG1 underlies a new subtype of autosomal recessive epidermolysis bullosa simplex
Groves RW, et al.
The Journal of Investigative Dermatology, 130(6), 1551-1557 (2010)
Structural analysis of the plakin domain of bullous pemphigoid antigen1 (BPAG1) suggests that plakins are members of the spectrin superfamily
Jefferson JJ, et al.
Journal of Molecular Biology, 366(1), 244-257 (2007)

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