Anti-Dystrophin Polyclonal Antibody
Product Sizes
100ug
£457.00
OAAI00793-100UG
About this Product
- SKU:
- OAAI00793
- Additional Names:
- BMD;CMD3B;DXS142;DXS164;DXS206;DXS230;DXS239;DXS268;DXS269;DXS270;DXS272;dystrophin;MRX85;truncated dystrophin.
- Clonality:
- Polyclonal
- Concentration:
- 100ug/200ul
- Extra Details:
- The dystrophin gene is the largest gene found in nature. The gene was identified throμgh 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. IHC staining of normal muscle tissue results in clear labeling confined to the periphery (plasma membrane) of normal muscle fibers. The product exhibits wide interspecies cross-reactivity.
- Gene Details:
- dystrophin
- Host:
- Rabbit
- Immunogen:
- A synthetic peptide corresponding to the intra domain 410-450aa of human Dystrophin protein. This sequence is identical to mouse and Pan troglodytes and other species.
- Isotype:
- Rabbit IgG
- Molecular Weight:
- >110
- Purification:
- Affinity Purified
- Shipping Conditions:
- Blue Ice
- Storage Conditions:
- The antibodies are stable for 24 months from date of receipt when stored at -20C to -70C. The antibodies can be stored at 2C-8C for three month without detectable loss of activity. Avoid repeated freezing-thawing cycles.
- Supplier:
- Aviva Systems Biology
- Type:
- Antibodies: Polyclonal Antibody
- Manufacturer's Data Sheet:html_datasheet.php
