With Or Without You: Premature Aging Whether Or Not Protein Is Modified

Main Category: Seniors / Aging
Also Included In: Biology / Biochemistry
Article Date: 04 Sep 2008 - 2:00 PDT

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Hutchinson-Gilford progeria syndrome (HGPS) is a rare childhood disease that resembles premature aging, and few affected children reach their teenage years. It is caused by a mutation in the LMNA gene that leads to the formation of progerin - a mutant form of the protein prelamin A. Once made, both progerin and normal prelamin A have the molecule farnesyl attached to them. This addition is later removed from normal prelamin A, whereas it cannot be removed from progerin. It has been suggested that farnesylated progerin is important for the development of disease in individuals with HGPS, and inhibitors of farnesylation are currently being evaluated in clinical trials. However, new data, generated by Stephen Young, Loren Fong, and colleagues, at the UCLA David Geffen School of Medicine, Los Angeles, in a new mouse model of HGPS suggest that inhibitors of farnesylation are likely to have limited therapeutic success as both farnesylated and nonfarnesylated progerin can cause symptoms of disease.

Notes:

TITLE: Progerin elicits disease phenotypes of progeria in mice whether or not it is farnesylated

AUTHORS:

Stephen G. Young
UCLA David Geffen School of Medicine, Los Angeles, California, USA.
http://www.mednet.ucla.edu

Loren G. Fong
UCLA David Geffen School of Medicine, Los Angeles, California, USA.
http://www.mednet.ucla.edu

View the PDF of this article at: https://www.the-jci.org/article.php?id=35876

Source:
Karen Honey
Journal of Clinical Investigation