DNA-damage accumulation and replicative arrest in Hutchinson–Gilford progeria syndrome
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چکیده
منابع مشابه
DNA-damage accumulation and replicative arrest in Hutchinson-Gilford progeria syndrome.
A common feature of progeria syndromes is a premature aging phenotype and an enhanced accumulation of DNA damage arising from a compromised repair system. HGPS (Hutchinson-Gilford progeria syndrome) is a severe form of progeria in which patients accumulate progerin, a mutant lamin A protein derived from a splicing variant of the lamin A/C gene (LMNA). Progerin causes chromatin perturbations whi...
متن کاملHutchinson-Gilford Progeria Syndrome
The Hutchinson-Gilford syndrome or progeria is a laminopathy generated by mutations that affect LMNA gene. This produces an abnormal protein named progerine which alters the formation of the cellular membrane inducing premature aging of all cells. In the present review aspects related to the pathophysiology and clinical characteristics of this syndrome are shown.
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ژورنال
عنوان ژورنال: Biochemical Society Transactions
سال: 2011
ISSN: 0300-5127,1470-8752
DOI: 10.1042/bst20110687