Review

Molecular ageing in progeroid syndromes: Hutchinson-Gilford progeria syndrome as a model

Henrique Douglas M Coutinho¹ , Vivyanne S Falcão-Silva² , Gregório Fernandes Gonçalves² and Raphael Batista da Nóbrega³

¹  Laboratório de Pesquisa em Produtos Naturais, Departamento de Ciências físicas e Biológicas, Centro de Ciências Biológicas e da Saúde, Universidade Regional do Cariri, Crato (CE), Brazil

²  Laboratório de Genética de Microrganismos, Departamento de Biologia Molecular, Centro de Ciências Exatas e da Natureza, Universidade Federal da Paraíba, João Pessoa (PB), Brazil

³  Laboratório de Bioquímica, Genética e Radiobiologia, Departamento de Biologia Molecular, Centro de Ciências Exatas e da Natureza, Universidade Federal da Paraíba, João Pessoa (PB), Brazil

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Immunity & Ageing 2009, 6:4doi:10.1186/1742-4933-6-4

Published:	20 April 2009

Abstract

Hutchinson-Gilford progeria syndrome (HGPS) is a rare premature aging disorder that belongs to a group of conditions called laminopathies which affect nuclear lamins. Mutations in two genes, LMNA and ZMPSTE24, have been found in patients with HGPS. The p.G608G LMNA mutation is the most commonly reported mutation. The aim of this work was to compile a comprehensive literature review of the clinical features and genetic mutations and mechanisms of this syndrome as a contribution to health care workers. This review shows the necessity of a more detailed clinical identification of Hutchinson-Gilford progeria syndrome and the need for more studies on the pharmacologic and pharmacogenomic approach to this syndrome.