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August 15, 1996The Journal of Cell Biology

Transgenic mdx mice expressing dystrophin with a deletion in the actin-binding domain display a "mild Becker" phenotype.

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Population

Transgenic mice lacking wild-type dystrophin (mdx)

Comparison

Expression of dystrophin deleted for the… vs Mice with deletions in the central rod domain…

Design

Preclinical

Authors

KCKathleen CorradoUniversity of North Carolina at Chapel HillJRJill A. RafaelUniversity of IowaPMPatti L. MillsNational Society of Genetic Counselors

Discussion

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Implication

High-level truncated dystrophin mitigates mdx phenotype in mice; leaves open relevance for human DMD gene therapy or cardiomyopathy management.

Structured PICO

P
Population
Transgenic mice lacking wild-type dystrophin (mdx)
I
Intervention
Expression of dystrophin deleted for the majority of the actin-binding domain (amino acids 45-273)
C
Comparator
Mice with deletions in the central rod domain, full-length dystrophin, or truncation of the COOH-terminal domain
O
Outcome
Dystrophic phenotype severitysurrogate

An intact actin-binding domain in dystrophin is not strictly essential to prevent severe muscular dystrophy in mdx mice, provided the truncated protein is expressed at high levels.

Cite This Study

Corrado et al. (1996) studied this question.

synapsesocial.com/papers/6a8a7b2106e1858e55de8ffchttps://doi.org/10.1083/jcb.134.4.873
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Also Consider

Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context:

  1. 1Searching for the 1 in 2,400,000: A review of dystrophin gene point mutations1994 · 161 citations
  2. 2Genetic and clinical correlations of Xp21 muscular dystrophy1992 · 64 citations
  3. 3Association of Aciculin with Dystrophin and Utrophin1995 · 53 citations
  4. 4Detailed analysis of the repeat domain of dystrophin reveals four potential hinge segments that may confer flexibility.1990 · 400 citations
  5. 5Length-tension relationship of mammalian diaphragm muscles1983 · 130 citations