DMD, Mouse, Clone: 3A11, Abnova™

Catalog No.	Quantity
89-021-332	100 μg

Catalog No. 89-021-332 Supplier Abnova Corporation Supplier No. H00001756M07

Description

Mouse monoclonal antibody raised against a full-length recombinant DMD.

The dystrophin gene is the largest gene found in nature, measuring 2.4Mb. 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 3500 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

Sequence: MREQLKGHETQTTCWDHPKMTELYQSLADLNNVRFSAYRTAMKLRRLQKALCLDLLSLSAACDALDQHNLKQNDQPMDILQIINCLTTIYDRLEQEHNNLVNVPLCVDMCLNWLLNVYDTGRTGRIRVLSFKTGIISLCKAHLEDKYRYLFKQVASSTGFCDQRRLGLLLHDSIQIPRQLGEVASFGGSNIEPSVRSCFQFANNKPEIEAALFLDWMRLEPQSMVWLPVLHRVAAAETAKHQAKCNICKECPIIGFRYRSLKHFNYDICQSCFFSGRVAKGHKMHYPMVEYCTPTTSGEDVRDFAKVLKNKFRTKRYFAKHPRMGYLPVQTVLEGDNMETPVTLINFWPVDSAPASSPQLSHDDTHSRIEHYASRLAEMENSNGSYLNDSISPNESIDDEHLLIQHYCQSLNQDSPLSQPRSPAQILISLESEERGELERILADLEEENRNLQAEYDRLKQQHEHKGLSPLPSPPEMMPTSPQSPRDAELIAEAKLLRQHKGRLEARMQILEDHNKQLESQLHRLRQLLEQPQAEAKVNGTTVSSPSTSLQRSDSSQPMLLRVVGSQTSDSMGEEDLLSPPQDTSTGLEEVMEQLNNSFPSSRGHNVGSLFHMADDLGRAMESLVSVMTDEEGAE

Specifications

• Antigen: DMD
• Applications: ELISA, Immunoprecipitation, Western Blot
• Classification: Monoclonal
• Clone: 3A11
• Conjugate: Unconjugated
• Description: Mouse monoclonal antibody raised against a full-length recombinant DMD.
• Formulation: PBS with no preservative; pH 7.4
• Gene: DMD
• Gene Accession No.: BC028720
• Gene Alias: BMD/CMD3B/DXS142/DXS164/DXS206/DXS230/DXS239/DXS268/DXS269/DXS270/DXS272
• Gene Symbols: DMD
• Host Species: Mouse
• Immunogen: 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.
• Purification Method: Affinity chromatography
• Quantity: 100 μg
• Regulatory Status: RUO
• Primary or Secondary: Primary
• Gene ID (Entrez): 1756
• Target Species: Human
• Content And Storage: Store at -20°C or lower. Aliquot to avoid repeated freezing and thawing.
• Product Type: Antibody
• Form: Liquid
• Isotype: IgG1 κ