Incomplete processing of mutant lamin A in Hutchinson-Gilford progeria leads to nuclear abnormalities, which are reversed by farnesyltransferase inhibition.
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The cell nucleus and aging: tantalizing clues and hopeful promisesMolecular ageing in progeroid syndromes: Hutchinson-Gilford progeria syndrome as a modelHutchinson-Gilford progeria mutant lamin A primarily targets human vascular cells as detected by an anti-Lamin A G608G antibody.Phenotype and course of Hutchinson-Gilford progeria syndromeBlocking protein farnesylation improves nuclear shape abnormalities in keratinocytes of mice expressing the prelamin A variant in Hutchinson-Gilford progeria syndromeModel of human aging: recent findings on Werner's and Hutchinson-Gilford progeria syndromesSevere mandibuloacral dysplasia-associated lipodystrophy and progeria in a young girl with a novel homozygous Arg527Cys LMNA mutationLamin A-dependent misregulation of adult stem cells associated with accelerated ageingA farnesyltransferase inhibitor prevents both the onset and late progression of cardiovascular disease in a progeria mouse modelThe nuclear envelope: an intriguing focal point for neurogenetic diseaseDesign of a microfluidic device to quantify dynamic intra-nuclear deformation during cell migration through confining environments.Analysis of prelamin A biogenesis reveals the nucleus to be a CaaX processing compartmentRecent advances in understanding the role of lamins in health and diseaseLaminopathies and the long strange trip from basic cell biology to therapyPrelamin A farnesylation and progeroid syndromesTemsirolimus Partially Rescues the Hutchinson-Gilford Progeria Cellular PhenotypeEmbryonic senescence and laminopathies in a progeroid zebrafish modelProtein farnesylation inhibitors cause donut-shaped cell nuclei attributable to a centrosome separation defectChemical inhibition of NAT10 corrects defects of laminopathic cells.Drug screening on Hutchinson Gilford progeria pluripotent stem cells reveals aminopyrimidines as new modulators of farnesylation.Involvement of xeroderma pigmentosum group A (XPA) in progeria arising from defective maturation of prelamin A.Genomic instability and DNA damage responses in progeria arising from defective maturation of prelamin A.Assessing the efficacy of protein farnesyltransferase inhibitors in mouse models of progeria.Lamin A rod domain mutants target heterochromatin protein 1alpha and beta for proteasomal degradation by activation of F-box protein, FBXW10Dynamics of lamin-A processing following precursor accumulation.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.DNA damage and laminsImpact of farnesylation inhibitors on survival in Hutchinson-Gilford progeria syndrome.An accumulation of non-farnesylated prelamin A causes cardiomyopathy but not progeria.Therapeutic intervention based on protein prenylation and associated modificationsDNA-damage accumulation and replicative arrest in Hutchinson-Gilford progeria syndromeFunctional coupling between the extracellular matrix and nuclear lamina by Wnt signaling in progeria.Requirements for efficient proteolytic cleavage of prelamin A by ZMPSTE24."Laminopathies": a wide spectrum of human diseases.Chromatin remodeling, DNA damage repair and aging.Prelamin A, Zmpste24, misshapen cell nuclei, and progeria--new evidence suggesting that protein farnesylation could be important for disease pathogenesis.Absence of progeria-like disease phenotypes in knock-in mice expressing a non-farnesylated version of progerin.Progressive vascular smooth muscle cell defects in a mouse model of Hutchinson-Gilford progeria syndrome
P2860
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P1343
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P2860
Incomplete processing of mutant lamin A in Hutchinson-Gilford progeria leads to nuclear abnormalities, which are reversed by farnesyltransferase inhibition.
description
2005 nî lūn-bûn
@nan
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
2005年论文
@zh
2005年论文
@zh-cn
name
Incomplete processing of mutan ...... arnesyltransferase inhibition.
@en
type
label
Incomplete processing of mutan ...... arnesyltransferase inhibition.
@en
prefLabel
Incomplete processing of mutan ...... arnesyltransferase inhibition.
@en
P4510
P356
P1476
Incomplete processing of mutan ...... arnesyltransferase inhibition.
@en
P2093
Michael W Glynn
Thomas W Glover
P304
P356
10.1093/HMG/DDI326
P577
2005-08-26T00:00:00Z