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Progerin-Induced Replication Stress Facilitates Premature Senescence in Hutchinson-Gilford Progeria Syndrome [PDF]

open access: yesMolecular and Cellular Biology, 2017
Hutchinson-Gilford progeria syndrome (HGPS) is caused by a mutation in LMNA that produces an aberrant lamin A protein, progerin. The accumulation of progerin in HGPS cells leads to an aberrant nuclear morphology, genetic instability, and p53-dependent premature senescence. How p53 is activated in response to progerin production is unknown. Here we show
Keith Wheaton   +2 more
exaly   +3 more sources
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Nucleocytoplasmic transport in cells with progerin-induced defective nuclear lamina

Biophysical Chemistry, 2017
Recent data indicate that nuclear lamina (NL) plays a relevant role in many fundamental cellular functions. The peculiar role of NL in cells is dramatically demonstrated by the Hutchinson-Gilford progeria syndrome (HGPS), an inherited laminopathy that causes premature, rapid aging shortly after birth.
Ferri G, Storti B, Bizzarri R
openaire   +4 more sources

Increased progerin expression associated with unusualLMNAmutations causes severe progeroid syndromes

Human Mutation, 2007
Hutchinson-Gilford progeria syndrome (HGPS) is a rare precocious aging syndrome caused by mutations in LMNA that lead to synthesis of a mutant form of prelamin A, generally called progerin, that cannot be processed to mature lamin A. Most HGPS patients have a recurrent heterozygous de novo mutation in exon 11 of LMNA, c.1824C>T/p.G608G; this synonymous
Moulson, Casey L.   +8 more
openaire   +3 more sources

Progerin - novi uzrok starenja stanica

2011
Prema današnjim spoznajama fiziološko se starenje stanice objašnjava progresivnim skraćivanjem telomernih dijelova DNA. U normalnim somatskim stanicama ovog enzima ima u minimalnim količinama pa su postupni gubitak telomera i posljedično starenje neizbježni. Nefiziološko starenje stanice mogu pokrenuti razni vanstanični ali i stanični poticaji, kao što
openaire   +1 more source

Increased progerin expression associated with unusualLMNAmutations causes severe progeroid syndromes

Human Mutation, 2007
Loren G Fong   +2 more
exaly  

Progerin and telomere dysfunction collaborate to trigger cellular senescence in normal human fibroblasts

Journal of Clinical Investigation, 2011
Kan Cao, Julia E Kieckhaefer
exaly  

Progerin elicits disease phenotypes of progeria in mice whether or not it is farnesylated

Journal of Clinical Investigation, 2008
Stephen G Young, Loren G Fong
exaly  

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