DMD, Mouse, Clone: DYS-48, Abnova™

Catalog No.	Quantity
89-110-437	100 μg

Catalog No. 89-110-437 Supplier Abnova Corporation Supplier No. MAB1453

Description

Mouse monoclonal antibody raised against full-length recombinant DMD.

The dystrophin gene is the largest gene found in nature, measuring 2.4Mb. 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 3500 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. [provided by RefSeq

Specifications

• Antigen: DMD
• Applications: Immunohistochemistry (Frozen), Western Blot
• Classification: Monoclonal
• Clone: MANDYS8
• Conjugate: Unconjugated
• Description: Mouse monoclonal antibody raised against full length recombinant DMD.
• Dilution: Western Blot (1-2 ug/mL) Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) (2-4 ug/mL) The optimal working dilution should be determined by the end user.
• Formulation: Lyophilized from 1.2% sodium acetate (2mg BSA, 0.01mg sodium azide)
• Gene: DMD
• Gene Alias: BMD/CMD3B/DXS142/DXS164/DXS206/DXS230/DXS239/DXS268/DXS269/DXS270/DXS272
• Gene Symbols: DMD
• Host Species: Mouse
• Immunogen: Recombinant protein corresponding to full length human DMD.
• Purification Method: Affinity purification
• Quantity: 100 μg
• Regulatory Status: RUO
• Primary or Secondary: Primary
• Gene ID (Entrez): 1756
• Target Species: Chicken, Human, Mouse, Rat
• Content And Storage: Store at -20°C on dry atmosphere. After reconstitution with 1mL of 1.2% sodium acetate or neutral PBS to a concentration of 100μg/mL store at -20°C or lower. Aliquot to avoid repeated freezing and thawing.
• Form: Lyophilized
• Isotype: IgG2b