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Annual Review of Genomics and Human Genetics

Volume 10, 2009

Review Article

The Posttranslational Processing of Prelamin A and Disease

Brandon S.J. Davies¹, Loren G. Fong¹, Shao H. Yang¹, Catherine Coffinier¹, and Stephen G. Young^1,2
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Affiliations: ¹Departments of Medicine, University of California, Los Angeles, California 90095; email: [email protected]; [email protected]; [email protected]; [email protected] ²Departments of Human Genetics, David Geffen School of Medicine, University of California, Los Angeles, California 90095; email: [email protected]
Vol. 10:153-174 (Volume publication date September 2009) https://doi.org/10.1146/annurev-genom-082908-150150
First published as a Review in Advance on May 19, 2009
© Annual Reviews

Abstract

Human geneticists have shown that some progeroid syndromes are caused by mutations that interfere with the conversion of farnesyl-prelamin A to mature lamin A. For example, Hutchinson-Gilford progeria syndrome is caused by LMNA mutations that lead to the accumulation of a farnesylated version of prelamin A. In this review, we discuss the posttranslational modifications of prelamin A and their relevance to the pathogenesis and treatment of progeroid syndromes.

Keyword(s): FTI, prenylation, restrictive dermopathy, ZMPSTE24

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2009-09-22

2024-12-08

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The Posttranslational Processing of Prelamin A and Disease

Annual Review of Genomics and Human Genetics 10, 153 (2009); https://doi.org/10.1146/annurev-genom-082908-150150

/content/journals/10.1146/annurev-genom-082908-150150

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Article Type: Review Article

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