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
| 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 MREQLKGHETQTTCWDHPKMTELYQ |
| 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 |

