Heterozygosity for Lmna deficiency eliminates the progeria-like phenotypes in Zmpste24-deficient mice
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Inhibiting farnesylation of progerin prevents the characteristic nuclear blebbing of Hutchinson-Gilford progeria syndromeTargeting protein prenylation in progeriaHuman ZMPSTE24 disease mutations: residual proteolytic activity correlates with disease severityBlocking protein farnesylation improves nuclear shape abnormalities in keratinocytes of mice expressing the prelamin A variant in Hutchinson-Gilford progeria syndromeWhen lamins go bad: nuclear structure and diseaseLaminopathies and the long strange trip from basic cell biology to therapyPrelamin A farnesylation and progeroid syndromesTRF2 and lamin A/C interact to facilitate the functional organization of chromosome endsZMPSTE24 defends against influenza and other pathogenic virusesFarnesylation of lamin B1 is important for retention of nuclear chromatin during neuronal migration.Dynamics of lamin-A processing following precursor accumulation.Reciprocal knock-in mice to investigate the functional redundancy of lamin B1 and lamin B2.Diseases of the nuclear envelope.The posttranslational processing of prelamin A and disease.Blocking protein farnesyltransferase improves nuclear blebbing in mouse fibroblasts with a targeted Hutchinson-Gilford progeria syndrome mutation.An accumulation of non-farnesylated prelamin A causes cardiomyopathy but not progeria.Blocking protein farnesyltransferase improves nuclear shape in fibroblasts from humans with progeroid syndromesDirect synthesis of lamin A, bypassing prelamin a processing, causes misshapen nuclei in fibroblasts but no detectable pathology in mice.Inhibiting farnesylation reverses the nuclear morphology defect in a HeLa cell model for Hutchinson-Gilford progeria syndrome.Aging and HIV/AIDS: pathogenetic role of therapeutic side effects"Laminopathies": a wide spectrum of human diseases.Cardiovascular pathology in Hutchinson-Gilford progeria: correlation with the vascular pathology of aging.Inner nuclear membrane proteins: impact on human disease.Homozygous and compound heterozygous mutations in ZMPSTE24 cause the laminopathy restrictive dermopathy.Chromatin remodeling, DNA damage repair and aging.Prelamin A and lamin A appear to be dispensable in the nuclear lamina.Accelerated ageing in mice deficient in Zmpste24 protease is linked to p53 signalling activation.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.Molecular bases of progeroid syndromes.A farnesyltransferase inhibitor improves disease phenotypes in mice with a Hutchinson-Gilford progeria syndrome mutation.Investigating the purpose of prelamin A processingType B mandibuloacral dysplasia with congenital myopathy due to homozygous ZMPSTE24 missense mutation.New ZMPSTE24 (FACE1) mutations in patients affected with restrictive dermopathy or related progeroid syndromes and mutation update.Systems genetics analysis of mouse chondrocyte differentiation.Mice that express farnesylated versions of prelamin A in neurons develop achalasia.'Relax and Repair' to restrain aging.Severe hepatocellular disease in mice lacking one or both CaaX prenyltransferases.MicroRNA transcriptome analysis identifies miR-365 as a novel negative regulator of cell proliferation in Zmpste24-deficient mouse embryonic fibroblasts.Mislocalization of prelamin A Tyr646Phe mutant to the nuclear pore complex in human embryonic kidney 293 cells.
P2860
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P2860
Heterozygosity for Lmna deficiency eliminates the progeria-like phenotypes in Zmpste24-deficient mice
description
2004 թուականի Դեկտեմբերին հրատարակուած գիտական յօդուած
@hyw
2004 թվականի դեկտեմբերին հրատարակված գիտական հոդված
@hy
article publié dans les Procee ...... f the United States of America
@fr
artículu científicu espublizáu en 2004
@ast
im Dezember 2004 veröffentlichter wissenschaftlicher Artikel
@de
scientific journal article
@en
vedecký článok (publikovaný 2004/12/28)
@sk
vědecký článek publikovaný v roce 2004
@cs
wetenschappelijk artikel (gepubliceerd op 2004/12/28)
@nl
наукова стаття, опублікована в грудні 2004
@uk
name
Heterozygosity for Lmna defici ...... pes in Zmpste24-deficient mice
@ast
Heterozygosity for Lmna defici ...... pes in Zmpste24-deficient mice
@en
Heterozygosity for Lmna defici ...... pes in Zmpste24-deficient mice
@nl
type
label
Heterozygosity for Lmna defici ...... pes in Zmpste24-deficient mice
@ast
Heterozygosity for Lmna defici ...... pes in Zmpste24-deficient mice
@en
Heterozygosity for Lmna defici ...... pes in Zmpste24-deficient mice
@nl
prefLabel
Heterozygosity for Lmna defici ...... pes in Zmpste24-deficient mice
@ast
Heterozygosity for Lmna defici ...... pes in Zmpste24-deficient mice
@en
Heterozygosity for Lmna defici ...... pes in Zmpste24-deficient mice
@nl
P2093
P2860
P3181
P356
P1476
Heterozygosity for Lmna defici ...... pes in Zmpste24-deficient mice
@en
P2093
Andrew Burghardt
Colin L Stewart
Jennifer K Ng
Loren G Fong
Margarita Meta
Martin O Bergo
Nathan Coté
Shao H Yang
Sharmila Majumdar
Terry Sullivan
P2860
P304
18111-18116
P3181
P356
10.1073/PNAS.0408558102
P407
P577
2004-12-17T00:00:00Z