DMD Antibody - C-terminal region
Produktgrößen
25ul
£177,00
ARP79548-P050-25UL
100 ul
£412,00
ARP79548-P050-100UL
Über dieses Produkt
- SKU:
- ARP79548-P050
- Zusätzliche Namen:
- BMD; CMD3B; MRX85; DXS142; DXS164; DXS206; DXS230; DXS239; DXS268; DXS269; DXS270; DXS272
- Klonalität:
- Polyclonal
- Konzentration:
- 0.5 mg/ml
- Weitere Details:
- 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.
- Gendetails:
- dystrophin
- Host:
- Rabbit
- Immunogen:
- The immunogen is a synthetic peptide directed towards the C terminal region of human DMD
- Molekulargewicht:
- 66 kDa
- Proteindetails:
- dystrophin
- Aufreinigung:
- Affinity purified
- Versandbedingungen:
- Blue Ice
- Lagerbedingungen:
- For short term use; store at 2-8C up to 1 week. For long term storage; store at -20C in small aliquots to prevent freeze-thaw cycles.
- Hersteller:
- Aviva Systems Biology
- Typ:
- Antibodies: Polyclonal Antibody
- Datenblatt des Herstellers:html_datasheet.php