Defective Lamin A-Rb Signaling in Hutchinson-Gilford Progeria Syndrome and Reversal by Farnesyltransferase Inhibition

Hutchinson-Gilford Progeria Syndrome (HGPS) is a rare premature aging disorder caused by a de novo heterozygous point mutation G608G (GGC>GGT) within exon 11 of LMNA gene encoding A-type nuclear lamins. This mutation elicits an internal deletion of 50 amino acids in the carboxyl-terminus of prela...

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Detalhes bibliográficos
Main Authors: Marji, Jackleen, O'Donoghue, Seán I., McClintock, Dayle, Satagopam, Venkata P., Schneider, Reinhard, Ratner, Desiree, J. Worman, Howard, Gordon, Leslie B., Djabali, Karima
Formato: Artigo
Idioma:Inglês
Publicado em: Public Library of Science 2010
Assuntos:
Research Article
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC2886113/
https://ncbi.nlm.nih.gov/pubmed/20559568
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1371/journal.pone.0011132
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