SAB1408565

Anti-Dystrophin Antibody

rabbit polyclonal

• 别名: BMD, CMD3B, DXS142, DXS164, DXS206, DXS230, DXS239, DXS268, DXS269
• 分類程式碼代碼: 12352203
• NACRES: NA.41

属性

• product name: Anti-DMD antibody produced in rabbit, purified immunoglobulin, buffered aqueous solution
• 生物源: rabbit
• 品質等級: 100
• 共軛: unconjugated
• 抗體表格: purified immunoglobulin
• 抗體產品種類: primary antibodies
• 無性繁殖: polyclonal
• 形狀: buffered aqueous solution
• 分子量: antigen ~72.2 kDa
• 物種活性: human
• 技術: western blot: 1 μg/mL
• NCBI登錄號: NM_004016.1
• UniProt登錄號: P11532
• 運輸包裝: dry ice
• 儲存溫度: −20°C
• 目標翻譯後修改: unmodified
• 基因資訊: human ... DMD(1756)

说明

一般說明

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

外觀

Solution in phosphate buffered saline, pH 7.4

安全信息

• 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