Dystrophin anticorps (C-Term)

N° du produit ABIN5515571

Cet anticorps anti-Dystrophin est un anticorps Lapin Polyclonal détectant Dystrophin dans WB. Adapté pour Humain.

Aperçu rapide pour Dystrophin anticorps (C-Term) (ABIN5515571)

Antigène: Dystrophin (DMD)

Reactivité: Humain

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp Dystrophin est non-conjugé

Application: Western Blotting (WB)

Détail du produit anti-Dystrophin anticorps

Épitope: C-Term

Séquence: SQTSDSMGEE DLLSPPQDTS TGLEEVMEQL NNSFPSSRGR NTPGKPMRED

Purification: Affinity purified

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

Information d'application

Indications d'application: Optimal working dilution should be determined by the investigator.

Restrictions: For Research Use only

Stockage

Format: Liquid

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

Agent conservateur: Sodium azide

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

Stock: -20 °C

Stockage commentaire: 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.

Détails sur Dystrophin

Antigène: Dystrophin (DMD)

Autre désignation: DMD

Sujet: 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

ID gène: 1756

NCBI Accession: NM_000109, NP_000100

Pathways: Skeletal Muscle Fiber Development