Dystrophin Antikörper (C-Term)

Produktnummer ABIN5515571

Dieses Anti-Dystrophin-Antikörper ist ein Kaninchen Polyklonal-Antikörper zur Detektion von Dystrophin in WB. Geeignet für Human.

Kurzübersicht für Dystrophin Antikörper (C-Term) (ABIN5515571)

Target: Dystrophin (DMD)

Reaktivität: Human

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser Dystrophin Antikörper ist unkonjugiert

Applikation: Western Blotting (WB)

Produktdetails anti-Dystrophin Antikörper

Bindungsspezifität: C-Term

Sequenz: SQTSDSMGEE DLLSPPQDTS TGLEEVMEQL NNSFPSSRGR NTPGKPMRED

Aufreinigung: Affinity purified

Immunogen: The immunogen is a synthetic peptide directed towards the C terminal region of human DMD

Anwendungsinformationen

Applikationshinweise: Optimal working dilution should be determined by the investigator.

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Buffer: Liquid. Purified antibody supplied in 1x PBS buffer with 0.09 % (w/v) sodium azide and 2 % sucrose.

Konservierungsmittel: Sodium azide

Vorsichtsmaßnahmen: This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Lagerung: -20 °C

Informationen zur Lagerung: For short term use, store at 2-8°C up to 1 week. For long term storage, store at -20°C in small aliquots to prevent freeze-thaw cycles.

Details zu Dystrophin

Target: Dystrophin (DMD)

Andere Bezeichnung: DMD

Hintergrund: 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.

Alias Symbols: BMD, CMD3B, MRX85, DXS142, DXS164, DXS206, DXS230, DXS239, DXS268, DXS269, DXS270, DXS272

Protein Size: 604

Gen-ID: 1756

NCBI Accession: NM_000109, NP_000100

Pathways: Skeletal Muscle Fiber Development