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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
价格
5045.46 *本价格含增值税费
促销
服务
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数量
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半岛bd体育手机客户端 名称:
Anti-DMD antibody produced in rabbit
CMD3B
DXS142
DXS164
DXS230
DXS268
BMD
DXS206
DXS239
DXS269
半岛bd体育手机客户端 介绍:

半岛bd体育手机客户端 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

NACRES NA.41

半岛bd体育手机客户端 性质

生物来源 rabbit
质量水平 100
偶联物 unconjugated
抗体形式 purified immunoglobulin
antibody product type primary antibodies
克隆 polyclonal
形式 buffered aqueous solution
分子量 antigen ~72.2 kDa
species reactivity human
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 Information human ... DMD(1756)

安全信息

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

Sigma-Aldrich

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