Dystrophin antibody (C-Term)

Catalog No. ABIN5515571

Product Details anti-Dystrophin Antibody

Target: Dystrophin (DMD)

Binding Specificity: C-Term

Reactivity: Human

Host: Rabbit

Clonality: Polyclonal

Conjugate: This Dystrophin antibody is un-conjugated

Application: Western Blotting (WB)

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 (DMD Products)

Synonyms: DMD antibody, Dmd antibody, MGC83347 antibody, DKFZp459C1629 antibody, dmd antibody, MGC79631 antibody, DKFZp468A1620 antibody, Dp71 antibody, cb664 antibody, zfDYS antibody, im:6911785 antibody, zgc:110165 antibody, BMD antibody, CMD3B antibody, DXS142 antibody, DXS164 antibody, DXS206 antibody, DXS230 antibody, DXS239 antibody, DXS268 antibody, DXS269 antibody, DXS270 antibody, DXS272 antibody, DXSmh7 antibody, DXSmh9 antibody, Dp427 antibody, dys antibody, mdx antibody, pke antibody, DNADMD1 antibody, RATDMD antibody, CG17750 antibody, CG31175 antibody, CG34157 antibody, CG7240 antibody, CG7243 antibody, CG7344 antibody, DLP antibody, DLP1 antibody, DLP186 antibody, DLP2 antibody, DLP3 antibody, DmDLP antibody, DmDYS antibody, Dmel\\CG34157 antibody, Dp117 antibody, Dp186 antibody, Dp205 antibody, GI3046716 antibody, IDLP antibody, det antibody, dmDLP antibody, dmDp186 antibody, dmDys antibody, dystrophin antibody, dystrophin, gene 1 L homeolog antibody, dystrophin, gene 1 antibody, dystrophin, muscular dystrophy antibody, Dystrophin antibody, DMD antibody, LOC708073 antibody, dmd.1.L antibody, LOC465557 antibody, dmd.1 antibody, dmd antibody, Dmd antibody, Dys antibody

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