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The Mutant Form of Lamin A that Causes Hutchinson-Gilford Progeria Is a Biomarker of Cellular Aging in Human Skin

Overview of attention for article published in PLOS ONE, December 2007
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Title
The Mutant Form of Lamin A that Causes Hutchinson-Gilford Progeria Is a Biomarker of Cellular Aging in Human Skin
Published in
PLOS ONE, December 2007
DOI 10.1371/journal.pone.0001269
Pubmed ID
Authors

Dayle McClintock, Desiree Ratner, Meepa Lokuge, David M. Owens, Leslie B. Gordon, Francis S. Collins, Karima Djabali

Abstract

Hutchinson-Gilford progeria syndrome (HGPS, OMIM 176670) is a rare disorder characterized by accelerated aging and early death, frequently from stroke or coronary artery disease. 90% of HGPS cases carry the LMNA G608G (GGC>GGT) mutation within exon 11 of LMNA, activating a splice donor site that results in production of a dominant negative form of lamin A protein, denoted progerin. Screening 150 skin biopsies from unaffected individuals (newborn to 97 years) showed that a similar splicing event occurs in vivo at a low level in the skin at all ages. While progerin mRNA remains low, the protein accumulates in the skin with age in a subset of dermal fibroblasts and in a few terminally differentiated keratinocytes. Progerin-positive fibroblasts localize near the basement membrane and in the papillary dermis of young adult skin; however, their numbers increase and their distribution reaches the deep reticular dermis in elderly skin. Our findings demonstrate that progerin expression is a biomarker of normal cellular aging and may potentially be linked to terminal differentiation and senescence in elderly individuals.

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Geographical breakdown

Country Count As %
Japan 3 1%
United States 2 <1%
Portugal 1 <1%
India 1 <1%
France 1 <1%
Canada 1 <1%
Finland 1 <1%
Unknown 263 96%

Demographic breakdown

Readers by professional status Count As %
Student > Ph. D. Student 64 23%
Student > Bachelor 54 20%
Researcher 42 15%
Student > Master 34 12%
Student > Doctoral Student 12 4%
Other 27 10%
Unknown 40 15%
Readers by discipline Count As %
Agricultural and Biological Sciences 88 32%
Biochemistry, Genetics and Molecular Biology 84 31%
Medicine and Dentistry 19 7%
Chemistry 6 2%
Pharmacology, Toxicology and Pharmaceutical Science 6 2%
Other 24 9%
Unknown 46 17%