Defective lamin A-Rb signaling in Hutchinson-Gilford Progeria Syndrome and reversal by farnesyltransferase inhibition.
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Molecular insights into the premature aging disease progeriaPotential therapeutic approaches for modulating expression and accumulation of defective lamin A in laminopathies and age-related diseasesTemsirolimus Partially Rescues the Hutchinson-Gilford Progeria Cellular PhenotypeRetinoblastoma protein prevents enteric nervous system defects and intestinal pseudo-obstruction.Higher-order unfolding of satellite heterochromatin is a consistent and early event in cell senescenceNuclear localization signal deletion mutants of lamin A and progerin reveal insights into lamin A processing and emerin targetingMitotic defects lead to pervasive aneuploidy and accompany loss of RB1 activity in mouse LmnaDhe dermal fibroblastsDiscordant gene expression signatures and related phenotypic differences in lamin A- and A/C-related Hutchinson-Gilford progeria syndrome (HGPS).Sulforaphane enhances progerin clearance in Hutchinson-Gilford progeria fibroblasts.Signaling pathway activation drift during aging: Hutchinson-Gilford Progeria Syndrome fibroblasts are comparable to normal middle-age and old-age cellsProliferation of progeria cells is enhanced by lamina-associated polypeptide 2α (LAP2α) through expression of extracellular matrix proteinsUnfolding the story of chromatin organization in senescent cells.A filtering strategy identifies FOXQ1 as a potential effector of lamin A dysfunction.Promoter hypermethylation as a mechanism for Lamin A/C silencing in a subset of neuroblastoma cells.Nuclear lamin functions and disease.Prelamin A causes progeria through cell-extrinsic mechanisms and prevents cancer invasion.Lamin A, farnesylation and agingLonafarnib for cancer and progeria.Hutchinson-Gilford progeria syndrome through the lens of transcription.Lamina-associated polypeptide (LAP)2α and nucleoplasmic lamins in adult stem cell regulation and disease.Methylene blue alleviates nuclear and mitochondrial abnormalities in progeria.Hutchinson-Gilford Progeria Syndrome: A premature aging disease caused by LMNA gene mutations.Progerin impairs chromosome maintenance by depleting CENP-F from metaphase kinetochores in Hutchinson-Gilford progeria fibroblasts.Phenotype-Dependent Coexpression Gene Clusters: Application to Normal and Premature Ageing.Phosphoinositide 3-kinase beta protects nuclear envelope integrity by controlling RCC1 localization and Ran activity.Protein sequestration at the nuclear periphery as a potential regulatory mechanism in premature aging.Lamin A and microtubules collaborate to maintain nuclear morphology.SENP1-modulated sumoylation regulates retinoblastoma protein (RB) and Lamin A/C interaction and stabilization.Autophagic Removal of Farnesylated Carboxy-Terminal Lamin Peptides.Skin and sural nerve biopsies: ultrastructural findings in the first genetically confirmed cases of CADASIL in Serbia.
P2860
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P2860
Defective lamin A-Rb signaling in Hutchinson-Gilford Progeria Syndrome and reversal by farnesyltransferase inhibition.
description
2010 nî lūn-bûn
@nan
2010 թուականի Յունիսին հրատարակուած գիտական յօդուած
@hyw
2010 թվականի հունիսին հրատարակված գիտական հոդված
@hy
2010年の論文
@ja
2010年論文
@yue
2010年論文
@zh-hant
2010年論文
@zh-hk
2010年論文
@zh-mo
2010年論文
@zh-tw
2010年论文
@wuu
name
Defective lamin A-Rb signaling ...... arnesyltransferase inhibition.
@ast
Defective lamin A-Rb signaling ...... arnesyltransferase inhibition.
@en
type
label
Defective lamin A-Rb signaling ...... arnesyltransferase inhibition.
@ast
Defective lamin A-Rb signaling ...... arnesyltransferase inhibition.
@en
prefLabel
Defective lamin A-Rb signaling ...... arnesyltransferase inhibition.
@ast
Defective lamin A-Rb signaling ...... arnesyltransferase inhibition.
@en
P2093
P2860
P1433
P1476
Defective lamin A-Rb signaling ...... arnesyltransferase inhibition.
@en
P2093
Dayle McClintock
Desiree Ratner
Howard J Worman
Jackleen Marji
Karima Djabali
Leslie B Gordon
Reinhard Schneider
Seán I O'Donoghue
P2860
P304
P356
10.1371/JOURNAL.PONE.0011132
P407
P577
2010-06-15T00:00:00Z