PROFESORES AL DÍA Educ. quím., 25(4), 432–439, 2014. © Universidad Nacional Autónoma de México, ISSN 0187-893-X Publicado en línea el 19 de agosto de 2014, ISSNE 1870-8404 Síndrome de Progeria de Hutchinson-Gilford. Causas, investigación y tratamientos...
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PROFESORES AL DÍA Educ. quím., 25(4), 432–439, 2014. © Universidad Nacional Autónoma de México, ISSN 0187-893-X Publicado en línea el 19 de agosto de 2014, ISSNE 1870-8404 Síndrome de Progeria de Hutchinson-Gilford. Causas, investigación y tratamientos farmacológicos María Genoveva González Morán* ABSTRACT (Hutchinson-Gilford Progeria Syndrome. Causes, research and pharmacological treatments) Hutchinson-Gilford progeria syndrome (HGPS) also known as childhood progeria is a rare genetic disease characterized by accelerated aging beginning in early childhood. The phenotypic features of this syndrome are caused by alterations in the lamin A protein, fibrillar component of the nuclear lamina which maintain the structure of the nuclear envelope and participates in organization chro- matin. Children with progeria have a point mutation in the LMNA gene which leads to the produc- tion of a permanently farnesylated mutant lamin A called progerin, that contribute to premature aging. In normal pr
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