SAB1408565

Anti-Dystrophin Antibody

rabbit polyclonal

• Sinónimos: BMD, CMD3B, DXS142, DXS164, DXS206, DXS230, DXS239, DXS268, DXS269
• Código UNSPSC: 12352203
• NACRES: NA.41

Propiedades

• Nombre del producto: Anti-DMD antibody produced in rabbit, purified immunoglobulin, buffered aqueous solution
• origen biológico: rabbit
• Nivel de calidad: 100
• conjugado: unconjugated
• forma del anticuerpo: purified immunoglobulin
• tipo de anticuerpo: primary antibodies
• clon: polyclonal
• Formulario: buffered aqueous solution
• mol peso: antigen ~72.2 kDa
• reactividad de especies: human
• técnicas: western blot: 1 μg/mL
• Nº de acceso NCBI: NM_004016.1
• Nº de acceso UniProt: P11532
• Condiciones de envío: dry ice
• temp. de almacenamiento: −20°C
• modificación del objetivo postraduccional: unmodified
• Información sobre el gen: human ... DMD(1756)

Descripción

Descripción general

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)

Inmunógeno

DMD (NP_004007.1, 1 a.a. ~ 635 a.a) full-length human protein.

Sequence
MREQLKGHETQTTCWDHPKMTELYQSLADLNNVRFSAYRTAMKLRRLQKALCLDLLSLSAACDALDQHNLKQNDQPMDILQIINCLTTIYDRLEQEHNNLVNVPLCVDMCLNWLLNVYDTGRTGRIRVLSFKTGIISLCKAHLEDKYRYLFKQVASSTGFCDQRRLGLLLHDSIQIPRQLGEVASFGGSNIEPSVRSCFQFANNKPEIEAALFLDWMRLEPQSMVWLPVLHRVAAAETAKHQAKCNICKECPIIGFRYRSLKHFNYDICQSCFFSGRVAKGHKMHYPMVEYCTPTTSGEDVRDFAKVLKNKFRTKRYFAKHPRMGYLPVQTVLEGDNMETPVTLINFWPVDSAPASSPQLSHDDTHSRIEHYASRLAEMENSNGSYLNDSISPNESIDDEHLLIQHYCQSLNQDSPLSQPRSPAQILISLESEERGELERILADLEEENRNLQAEYDRLKQQHEHKGLSPLPSPPEMMPTSPQSPRDAELIAEAKLLRQHKGRLEARMQILEDHNKQLESQLHRLRQLLEQPQAEAKVNGTTVSSPSTSLQRSDSSQPMLLRVVGSQTSDSMGEEDLLSPPQDTSTGLEEVMEQLNNSFPSSRGHNVGSLFHMADDLGRAMESLVSVMTDEEGAE

Forma física

Solution in phosphate buffered saline, pH 7.4

Información de seguridad

• Código de clase de almacenamiento: 10 - Combustible liquids
• Clase de riesgo para el agua (WGK): WGK 1
• Punto de inflamabilidad (°F): Not applicable
• Punto de inflamabilidad (°C): Not applicable