Dystrophin (2765-2777)

  • Description

  • Application Data

Description

Dystrophin (2765-2777) has been tested as a peptide for accurate quantification of dystrophin levels in skeletal biopsies. Levels of this fragment could be used to accurately assess the progress of dystrophin treatment of Duchenne muscular dystrophy (DMD) patients.

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Application Data

Catalogue number crb1001662
Molecular Weight 1401.7
Sequence (three letter code)

H-Ser-Leu-Glu-Gly-Ser-Asp-Asp-Ala-Val-Leu-Leu-Gln-Arg-acid

Storage -20°C
Citations

Gao and McNally (2015). The Dystrophin Complex: structure, function and implications for therapy. Compr. Physiol., 5(3): 1223. PMID: 26140716.

 

Farrokhi et al., (2021). Dystrophin and mini-dystrophin quantification by mass spectrometry in skeletal muscle for gene therapy development in Duchenne muscular dystrophy. Gene Ther. doi: 10.1038/s41434-021-00300-7. 

References

Gao and McNally (2015). The Dystrophin Complex: structure, function and implications for therapy. Compr. Physiol., 5(3): 1223. PMID: 26140716.

 

Farrokhi et al., (2021). Dystrophin and mini-dystrophin quantification by mass spectrometry in skeletal muscle for gene therapy development in Duchenne muscular dystrophy. Gene Ther. doi: 10.1038/s41434-021-00300-7. 

Data Sheet Material Safety Data Sheet (MSDS)

Forms of inherited muscular dystrophy such as Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD) result from mutations targeting the dystrophin gene. These disorders are X-linked, progressive, and cause the gradual weakening of the muscles leading to respiratory failure and ultimately reduces the patient’s lifespan.

In DMD, mutations lead to the production of premature stop codons and hence the truncated dystrophin protein product is vulnerable to nonsense mediated decay and degradation. Therefore, dystrophin production in muscle cells is reduced. On the other hand, nonsense mutations which also contribute to DMD, cause exon skipping in BMD and result in an internally truncated protein product which are partially functional. The symptoms of BMD are later onset compared with DMD which develop in patients between 2 to 7 years.

Treatments of dystrophin disorders are in clinical trial including antisense oligonucleotide exon skipping and gene therapy. However, the efficacies of these treatments are not easily quantified. Currently levels of muscular dystrophin are quantified by western blot which can be unreliable. The peptide provided here, aligning residues dystrophin (2690-2700), has been tested via mass spectrometry to provide a more reliable method of validation of dystrophin levels. Further study with this dystrophin fragment could prove to be a vital step in the understanding and treatment of dystrophin disorders. Within our catalogue we also have other peptides tested for dystrophin quantification available plus the full-length dystrophin protein.

Dystrophin (2765-2777)

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£85.00
£110.00
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