Epidermal expression of the truncated prelamin A causing Hutchinson-Gilford progeria syndrome: effects on keratinocytes, hair and skin
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Molecular ageing in progeroid syndromes: Hutchinson-Gilford progeria syndrome as a modelBlocking protein farnesylation improves nuclear shape abnormalities in keratinocytes of mice expressing the prelamin A variant in Hutchinson-Gilford progeria syndromeMouse models of laminopathiesLaminopathies and the long strange trip from basic cell biology to therapyA molecular mechanism underlying the neural-specific defect in torsinA mutant miceExpression of progerin in aging mouse brains reveals structural nuclear abnormalities without detectible significant alterations in gene expression, hippocampal stem cells or behavior.Defective lamin A-Rb signaling in Hutchinson-Gilford Progeria Syndrome and reversal by farnesyltransferase inhibition.Diseases of the nuclear envelope.The posttranslational processing of prelamin A and disease.Discordant gene expression signatures and related phenotypic differences in lamin A- and A/C-related Hutchinson-Gilford progeria syndrome (HGPS).Direct synthesis of lamin A, bypassing prelamin a processing, causes misshapen nuclei in fibroblasts but no detectable pathology in mice.Cell autonomous and systemic factors in progeria development.Knock-down of methyl CpG-binding protein 2 (MeCP2) causes alterations in cell proliferation and nuclear lamins expression in mammalian cells.Speeding up the clock: The past, present and future of progeria.Nuclear lamina defects cause ATM-dependent NF-κB activation and link accelerated aging to a systemic inflammatory response.Blocking farnesylation of the prelamin A variant in Hutchinson-Gilford progeria syndrome alters the distribution of A-type lamins.Accelerated aging syndromes, are they relevant to normal human aging?Disrupting the LINC complex in smooth muscle cells reduces aortic disease in a mouse model of Hutchinson-Gilford progeria syndrome
P2860
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P2860
Epidermal expression of the truncated prelamin A causing Hutchinson-Gilford progeria syndrome: effects on keratinocytes, hair and skin
description
article científic
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article scientifique
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articolo scientifico
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artigo científico
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bilimsel makale
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scientific article published on 28 April 2008
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vedecký článok
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vetenskaplig artikel
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videnskabelig artikel
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vědecký článek
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name
Epidermal expression of the tr ...... n keratinocytes, hair and skin
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Epidermal expression of the tr ...... keratinocytes, hair and skin.
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type
label
Epidermal expression of the tr ...... n keratinocytes, hair and skin
@en
Epidermal expression of the tr ...... keratinocytes, hair and skin.
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prefLabel
Epidermal expression of the tr ...... n keratinocytes, hair and skin
@en
Epidermal expression of the tr ...... keratinocytes, hair and skin.
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P2093
P2860
P356
P1476
Epidermal expression of the tr ...... n keratinocytes, hair and skin
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P2093
Colin L Stewart
David M Owens
Howard J Worman
Karima Djabali
Yuexia Wang
P2860
P304
P356
10.1093/HMG/DDN136
P577
2008-04-28T00:00:00Z