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Anti-DMD antibody produced in rabbit

品牌
Sigma-Aldrich
货号
SAB1408565
规格纯度
purified immunoglobulin, buffered aqueous solution
参考价格
5045.46 *本价格含增值税费
促销
服务
  • 原厂保证
  • 包邮
  • 增值税票
数量
-+
产品名称:
Anti-DMD antibody produced in rabbit
CMD3B
DXS142
DXS164
DXS230
DXS268
BMD
DXS206
DXS239
DXS269
产品介绍:

产品说明

一般描述

The dystrophin gene is the largest gene found in nature, measuring 2.4 Mb. The gene was identified through a positional cloning approach, targeted at the isolation of the gene responsible for Duchenne (DMD) and Becker (BMD) Muscular Dystrophies. DMD is a recessive, fatal, X-linked disorder occurring at a frequency of about 1 in 3,500 new-born males. BMD is a milder allelic form. In general, DMD patients carry mutations which cause premature translation termination (nonsense or frame shift mutations), while in BMD patients dystrophin is reduced either in molecular weight (derived from in-frame deletions) or in expression level. The dystrophin gene is highly complex, containing at least eight independent, tissue-specific promoters and two polyA-addition sites. Furthermore, dystrophin RNA is differentially spliced, producing a range of different transcripts, encoding a large set of protein isoforms. Dystrophin (as encoded by the Dp427 transcripts) is a large, rod-like cytoskeletal protein which is found at the inner surface of muscle fibers. Dystrophin is part of the dystrophin-glycoprotein complex (DGC), which bridges the inner cytoskeleton (F-actin) and the extra-cellular matrix. (provided by RefSeq)

免疫原

DMD (NP_004007.1, 1 a.a. ~ 635 a.a) full-length human protein.

Sequence
MREQLKGHETQTTCWDHPKMTELYQSLADLNNVRFSAYRTAMKLRRLQKALCLDLLSLSAACDALDQHNLKQNDQPMDILQIINCLTTIYDRLEQEHNNLVNVPLCVDMCLNWLLNVYDTGRTGRIRVLSFKTGIISLCKAHLEDKYRYLFKQVASSTGFCDQRRLGLLLHDSIQIPRQLGEVASFGGSNIEPSVRSCFQFANNKPEIEAALFLDWMRLEPQSMVWLPVLHRVAAAETAKHQAKCNICKECPIIGFRYRSLKHFNYDICQSCFFSGRVAKGHKMHYPMVEYCTPTTSGEDVRDFAKVLKNKFRTKRYFAKHPRMGYLPVQTVLEGDNMETPVTLINFWPVDSAPASSPQLSHDDTHSRIEHYASRLAEMENSNGSYLNDSISPNESIDDEHLLIQHYCQSLNQDSPLSQPRSPAQILISLESEERGELERILADLEEENRNLQAEYDRLKQQHEHKGLSPLPSPPEMMPTSPQSPRDAELIAEAKLLRQHKGRLEARMQILEDHNKQLESQLHRLRQLLEQPQAEAKVNGTTVSSPSTSLQRSDSSQPMLLRVVGSQTSDSMGEEDLLSPPQDTSTGLEEVMEQLNNSFPSSRGHNVGSLFHMADDLGRAMESLVSVMTDEEGAE

外形

Solution in phosphate buffered saline, pH 7.4

基本信息

NACRESNA.41

产品性质

生物来源rabbit
质量水平100
偶联物unconjugated
抗体形式purified immunoglobulin
antibody product typeprimary antibodies
克隆polyclonal
形式buffered aqueous solution
分子量antigen ~72.2 kDa
species reactivityhuman
technique(s)western blot: 1 μg/mL
NCBI登记号NM_004016.1
UniProt登记号P11532
运输dry ice
储存温度−20℃
Gene Informationhuman ... DMD(1756)

安全信息

储存分类代码13 - Non Combustible Solids
WGKWGK 1
闪点(F)Not applicable
闪点(C)Not applicable

Sigma-Aldrich

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