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Monoclonal Anti-DMD antibody produced in mouse
| 产品编号： | 3144187 |
| 规格： | clone 3A11, purified immunoglobulin, buffered aqueous solution |
| 包装规格： | 100 μG |
| 产品类别： | 进口试剂 |
| 品牌： | Sigma-Aldrich |
| 优惠价： | 立即咨询 |
产品别名
Monoclonal Anti-DMD antibody produced in mouse
CMD3B
DXS142
DXS164
DXS230
DXS268
BMD
DXS206
DXS239
DXS269
基本信息
| NACRES | NA.41 |
| General description【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) |
| Immunogen【免疫原】 | DMD (AAH28720.1, 1 a.a. ~ 635 a.a) full-length recombinant protein with GST tag. MW of the GST tag alone is 26 KDa. Sequence MREQLKGHETQTTCWDHPKMTELYQSLADLNNVRFSAYRTAMKLRRLQKALCLDLLSLSAACDALDQHNLKQNDQPMDILQIINCLTTIYDRLEQEHNNLVNVPLCVDMCLNWLLNVYDTGRTGRIRVLSFKTGIISLCKAHLEDKYRYLFKQVASSTGFCDQRRLGLLLHDSIQIPRQLGEVASFGGSNIEPSVRSCFQFANNKPEIEAALFLDWMRLEPQSMVWLPVLHRVAAAETAKHQAKCNICKECPIIGFRYRSLKHFNYDICQSCFFSGRVAKGHKMHYPMVEYCTPTTSGEDVRDFAKVLKNKFRTKRYFAKHPRMGYLPVQTVLEGDNMETPVTLINFWPVDSAPASSPQLSHDDTHSRIEHYASRLAEMENSNGSYLNDSISPNESIDDEHLLIQHYCQSLNQDSPLSQPRSPAQILISLESEERGELERILADLEEENRNLQAEYDRLKQQHEHKGLSPLPSPPEMMPTSPQSPRDAELIAEAKLLRQHKGRLEARMQILEDHNKQLESQLHRLRQLLEQPQAEAKVNGTTVSSPSTSLQRSDSSQPMLLRVVGSQTSDSMGEEDLLSPPQDTSTGLEEVMEQLNNSFPSSRGHNVGSLFHMADDLGRAMESLVSVMTDEEGAE |
| Physical form【外形】 | Solution in phosphate buffered saline, pH 7.4 |
产品性质
| Quality Level【质量水平】 | 100 |
| biological source【生物来源】 | mouse |
| conjugate【偶联物】 | unconjugated |
| antibody form【抗体形式】 | purified immunoglobulin |
| antibody product type | primary antibodies |
| clone【克隆】 | 3A11, monoclonal |
| form【形式】 | buffered aqueous solution |
| mol wt【分子量】 | antigen ~95.96 kDa |
| species reactivity | human |
| technique(s) | immunoprecipitation (IP): suitable indirect ELISA: suitable western blot: 1-5 μg/mL |
| isotype【同位素/亚型】 | IgG1κ |
| NCBI accession no.【NCBI登记号】 | BC028720 |
| UniProt accession no.【UniProt登记号】 | P11532 |
| shipped in【运输】 | dry ice |
| storage temp.【储存温度】 | −20℃ |
| Gene Information | human ... DMD(1756) |
安全信息
| Storage Class Code【储存分类代码】 | 13 - Non Combustible Solids |
| WGK | WGK 1 |
