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HPA030200

Sigma-Aldrich

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

同義詞:

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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About This Item

分類程式碼代碼:
12352203
人類蛋白質圖譜編號:
NACRES:
NA.41

生物源

rabbit

共軛

unconjugated

抗體表格

affinity isolated antibody

抗體產品種類

primary antibodies

無性繁殖

polyclonal

產品線

Prestige Antibodies® Powered by Atlas Antibodies

形狀

buffered aqueous glycerol solution

物種活性

human

技術

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

免疫原序列

KPRCQNVAEDMDIQLCASILNENSDENENINTMILLDKMHSCSSLEKQQRVNVVQLASPSENNLVTEKSNLPEYTTEIAGKSKENLLNHE

UniProt登錄號

運輸包裝

wet ice

儲存溫度

−20°C

目標翻譯後修改

unmodified

基因資訊

human ... DST(667)

一般說明

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)

應用

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.

特點和優勢

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.

聯結

Corresponding Antigen APREST77625

外觀

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

法律資訊

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

免責聲明

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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儲存類別代碼

10 - Combustible liquids

水污染物質分類(WGK)

WGK 1

閃點(°F)

Not applicable

閃點(°C)

Not applicable


分析證明 (COA)

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存取文件庫

Novel mutations in dystonin provide clues to the pathomechanisms of HSAN-VI
Manganelli F, et al.
Neurology, 88(22), 2132-2140 (2017)
Hereditary sensory autonomic neuropathy caused by a mutation in dystonin
Edvardson S, et al.
Annals of Neurology, 71(4), 569-572 (2012)
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)
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)
Cloning and characterization of the neural isoforms of human dystonin
Brown A, et al.
Genomics, 29(3), 777-780 (1995)

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