Dystrophin antibody (C-Term)

Catalog No. ABIN5515571

The Rabbit Polyclonal anti-Dystrophin antibody (ABIN5515571) specifically detects Dystrophin in WB. The antibody is reactive with Human samples.

Quick Overview for Dystrophin antibody (C-Term) (ABIN5515571)

Target: Dystrophin (DMD)

Reactivity: Human

Host: Rabbit

Clonality: Polyclonal

Conjugate: This Dystrophin antibody is un-conjugated

Application: Western Blotting (WB)

Product Details anti-Dystrophin Antibody

Binding Specificity: C-Term

Sequence: SQTSDSMGEE DLLSPPQDTS TGLEEVMEQL NNSFPSSRGR NTPGKPMRED

Purification: Affinity purified

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

Application Details

Application Notes: Optimal working dilution should be determined by the investigator.

Restrictions: For Research Use only

Handling

Format: Liquid

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

Preservative: Sodium azide

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

Storage: -20 °C

Storage Comment: 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.

Target Details for Dystrophin

Target: Dystrophin (DMD)

Alternative Name: DMD

Background: 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

Gene ID: 1756

NCBI Accession: NM_000109, NP_000100

Pathways: Skeletal Muscle Fiber Development