Dermal fibroblasts in Hutchinson-Gilford progeria syndrome with the lamin A G608G mutation have dysmorphic nuclei and are hypersensitive to heat stress
about
The mutant form of lamin A that causes Hutchinson-Gilford progeria is a biomarker of cellular aging in human skinHutchinson-Gilford progeria mutant lamin A primarily targets human vascular cells as detected by an anti-Lamin A G608G antibody.Blocking protein farnesylation improves nuclear shape abnormalities in keratinocytes of mice expressing the prelamin A variant in Hutchinson-Gilford progeria syndromeTemsirolimus Partially Rescues the Hutchinson-Gilford Progeria Cellular PhenotypeFibroblast phenotype in male carriers of FMR1 premutation allelesDiseases of the nuclear envelope.Genomic features defining exonic variants that modulate splicing.Discordant gene expression signatures and related phenotypic differences in lamin A- and A/C-related Hutchinson-Gilford progeria syndrome (HGPS)."Laminopathies": a wide spectrum of human diseases.Biology of the Mi-2/NuRD Complex in SLAC (Stemness, Longevity/Ageing, and Cancer).Mutations in LMNA modulate the lamin A--Nesprin-2 interaction and cause LINC complex alterationsMechanics of the nucleus.Concentration-dependent Effects of Nuclear Lamins on Nuclear Size in Xenopus and Mammalian Cells.Insights into the role of immunosenescence during varicella zoster virus infection (shingles) in the aging cell modelHutchinson-Gilford progeria syndrome: oral and craniofacial phenotypes.Epidermal expression of the truncated prelamin A causing Hutchinson-Gilford progeria syndrome: effects on keratinocytes, hair and skinCan we intervene in human ageing?Induced pluripotent stem cells reveal functional differences between drugs currently investigated in patients with hutchinson-gilford progeria syndrome.In vitro pathological modelling using patient-specific induced pluripotent stem cells: the case of progeria.Blocking farnesylation of the prelamin A variant in Hutchinson-Gilford progeria syndrome alters the distribution of A-type lamins.Progerin impairs chromosome maintenance by depleting CENP-F from metaphase kinetochores in Hutchinson-Gilford progeria fibroblasts.Implant supported prosthesis in a patient with progeria: case report.Naïve adult stem cells from patients with Hutchinson-Gilford progeria syndrome express low levels of progerin in vivo.Intermittent treatment with farnesyltransferase inhibitor and sulforaphane improves cellular homeostasis in Hutchinson-Gilford progeria fibroblasts.Age of heart disease presentation and dysmorphic nuclei in patients with LMNA mutations.Autophagic Removal of Farnesylated Carboxy-Terminal Lamin Peptides.Molecular functional analyses revealed essential roles of HSP90 and lamin A/C in growth, migration, and self-aggregation of dermal papilla cells.
P2860
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P2860
Dermal fibroblasts in Hutchinson-Gilford progeria syndrome with the lamin A G608G mutation have dysmorphic nuclei and are hypersensitive to heat stress
description
2005 nî lūn-bûn
@nan
2005 թուականին հրատարակուած գիտական յօդուած
@hyw
2005 թվականին հրատարակված գիտական հոդված
@hy
2005年の論文
@ja
2005年論文
@yue
2005年論文
@zh-hant
2005年論文
@zh-hk
2005年論文
@zh-mo
2005年論文
@zh-tw
2005年论文
@wuu
name
Dermal fibroblasts in Hutchins ...... hypersensitive to heat stress
@ast
Dermal fibroblasts in Hutchins ...... hypersensitive to heat stress
@en
Dermal fibroblasts in Hutchins ...... hypersensitive to heat stress
@en-gb
Dermal fibroblasts in Hutchins ...... hypersensitive to heat stress
@nl
type
label
Dermal fibroblasts in Hutchins ...... hypersensitive to heat stress
@ast
Dermal fibroblasts in Hutchins ...... hypersensitive to heat stress
@en
Dermal fibroblasts in Hutchins ...... hypersensitive to heat stress
@en-gb
Dermal fibroblasts in Hutchins ...... hypersensitive to heat stress
@nl
prefLabel
Dermal fibroblasts in Hutchins ...... hypersensitive to heat stress
@ast
Dermal fibroblasts in Hutchins ...... hypersensitive to heat stress
@en
Dermal fibroblasts in Hutchins ...... hypersensitive to heat stress
@en-gb
Dermal fibroblasts in Hutchins ...... hypersensitive to heat stress
@nl
P2093
P2860
P3181
P356
P1433
P1476
Dermal fibroblasts in Hutchins ...... hypersensitive to heat stress
@en
P2093
Christina Pedicelli
Dayle McClintock
Howard J Worman
Karima Djabali
Mauro Paradisi
Revekka L Boguslavsky
P2860
P2888
P3181
P356
10.1186/1471-2121-6-27
P407
P577
2005-01-01T00:00:00Z
P5875
P6179
1022857206