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Anti-DMD antibody produced in rabbit

SIGMA/SAB1408565 - purified immunoglobulin, buffered aqueous solution

Synonym: BMD; CMD3B; DXS142; DXS164; DXS206; DXS230; DXS239; DXS268; DXS269

Product Type: Chemical

Catalog Number PKG Qty. Price Quantity
45-SAB1408565-100UG 100 µg
$580.00
1/EA
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Western Blotting Western Blot analysis of DMD expression in transfected 293T cell line by DMD polyclonal antibody. Lanes Lane 1: DMD transfected lysate (72.20 kDa). Lane 2: Non-transfected lysate.

 

antibody form purified immunoglobulin
antibody product type primary antibodies
biological source rabbit
clone polyclonal
conjugate unconjugated
form buffered aqueous solution
mol wt antigen ~72.2 kDa
NCBI accession no. NM_004016.1 
Quality Level 100 
shipped in dry ice
species reactivity human
storage temp. −20°C
target post-translational modification unmodified
technique(s) western blot: 1 μg/mL
UniProt accession no. P11532 
General description: 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: DMD (NP_004007.1, 1 a.a. ~ 635 a.a) full-length human protein.

Sequence
MREQLKGHETQTTCWDHPKMTELYQSLADLNNVRFSAYRTAMKLRRLQKALCLDLLSLSAACDALDQHNLKQNDQPMDILQIINCLTTIYDRLEQEHNNLVNVPLCVDMCLNWLLNVYDTGRTGRIRVLSFKTGIISLCKAHLEDKYRYLFKQVASSTGFCDQRRLGLLLHDSIQIPRQLGEVASFGGSNIEPSVRSCFQFANNKPEIEAALFLDWMRLEPQSMVWLPVLHRVAAAETAKHQAKCNICKECPIIGFRYRSLKHFNYDICQSCFFSGRVAKGHKMHYPMVEYCTPTTSGEDVRDFAKVLKNKFRTKRYFAKHPRMGYLPVQTVLEGDNMETPVTLINFWPVDSAPASSPQLSHDDTHSRIEHYASRLAEMENSNGSYLNDSISPNESIDDEHLLIQHYCQSLNQDSPLSQPRSPAQILISLESEERGELERILADLEEENRNLQAEYDRLKQQHEHKGLSPLPSPPEMMPTSPQSPRDAELIAEAKLLRQHKGRLEARMQILEDHNKQLESQLHRLRQLLEQPQAEAKVNGTTVSSPSTSLQRSDSSQPMLLRVVGSQTSDSMGEEDLLSPPQDTSTGLEEVMEQLNNSFPSSRGHNVGSLFHMADDLGRAMESLVSVMTDEEGAE
Physical form: Solution in phosphate buffered saline, pH 7.4
RIDADR NONH for all modes of transport
WGK Germany WGK 1
Flash Point(F) Not applicable
Flash Point(C) Not applicable
Storage Temp. −20°C
UNSPSC 12352203

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