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Human Molecular Genetics, 1999, Vol. 8, No. 10 1847-1851
© 1999 Oxford University Press

Review

Heart to heart: from nuclearproteins to Emery–Dreifuss muscular dystrophy

Glenn E. Morrisa and Sushila Manilal

MRIC Biochemistry Group, NE Wales Institute, Mold Road,Wrexham LL11 2AW, UK

ABSTRACT

Emery–Dreifuss muscular dystrophy has someremarkably specific features, with only cardiac and skeletal tissuesbeing affected. Equally remarkably, the disease is caused by mutationsin widely expressed genes for the nuclear membrane/laminaproteins, emerin and lamin A/C. How do mutations in proteinsat the heart of the cell lead to stiff joints and sudden heart failure?This and related questions are the subject of this review.

FOOTNOTES

a Towhom correspondence should be addressed. Tel: +44 1978293330; Fax: +44 1978 290008; Email: morrisge{at}newi.ac.uk


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