Blocking protein farnesyltransferase improves nuclear shape in fibroblasts from humans with progeroid syndromes - Wikidata - awgldk - TriplyDB

Blocking protein farnesyltransferase improves nuclear shape in fibroblasts from humans with progeroid syndromes

Blocking protein farnesyltransferase improves nuclear shape in fibroblasts from humans with progeroid syndromes

http://www.wikidata.org/entity/Q33930224

about

The cell nucleus and aging: tantalizing clues and hopeful promises The mutant form of lamin A that causes Hutchinson-Gilford progeria is a biomarker of cellular aging in human skin Molecular ageing in progeroid syndromes: Hutchinson-Gilford progeria syndrome as a model Hutchinson-Gilford progeria mutant lamin A primarily targets human vascular cells as detected by an anti-Lamin A G608G antibody.Progeria: a rare genetic premature ageing disorder Targeting protein prenylation in progeria Blocking protein farnesylation improves nuclear shape abnormalities in keratinocytes of mice expressing the prelamin A variant in Hutchinson-Gilford progeria syndrome Model of human aging: recent findings on Werner's and Hutchinson-Gilford progeria syndromes A farnesyltransferase inhibitor prevents both the onset and late progression of cardiovascular disease in a progeria mouse model Molecular insights into the premature aging disease progeria The nuclear envelope: an intriguing focal point for neurogenetic disease Analysis of prelamin A biogenesis reveals the nucleus to be a CaaX processing compartment Recent advances in understanding the role of lamins in health and disease Laminopathies and the long strange trip from basic cell biology to therapy Prelamin A farnesylation and progeroid syndromes Embryonic senescence and laminopathies in a progeroid zebrafish model A progeria mutation reveals functions for lamin A in nuclear assembly, architecture, and chromosome organization.Farnesylated nuclear proteins Kugelkern and lamin Dm0 affect nuclear morphology by directly interacting with the nuclear membrane.Protein farnesylation inhibitors cause donut-shaped cell nuclei attributable to a centrosome separation defect Chemical inhibition of NAT10 corrects defects of laminopathic cells.Involvement of xeroderma pigmentosum group A (XPA) in progeria arising from defective maturation of prelamin A.Assessing the efficacy of protein farnesyltransferase inhibitors in mouse models of progeria.Dynamics of lamin-A processing following precursor accumulation.Defective lamin A-Rb signaling in Hutchinson-Gilford Progeria Syndrome and reversal by farnesyltransferase inhibition.Nuclear localization signal deletion mutants of lamin A and progerin reveal insights into lamin A processing and emerin targeting Targeting protein prenylation for cancer therapy Diseases of the nuclear envelope.Genetic studies on the functional relevance of the protein prenyltransferases in skin keratinocytes.The posttranslational processing of prelamin A and disease.DNA damage and lamins Impact of farnesylation inhibitors on survival in Hutchinson-Gilford progeria syndrome.An accumulation of non-farnesylated prelamin A causes cardiomyopathy but not progeria.Metformin decreases progerin expression and alleviates pathological defects of Hutchinson-Gilford progeria syndrome cells.Therapeutic intervention based on protein prenylation and associated modifications Direct synthesis of lamin A, bypassing prelamin a processing, causes misshapen nuclei in fibroblasts but no detectable pathology in mice.Role of progerin-induced telomere dysfunction in HGPS premature cellular senescence.Functional coupling between the extracellular matrix and nuclear lamina by Wnt signaling in progeria.The role of DNA damage in laminopathy progeroid syndromes."Laminopathies": a wide spectrum of human diseases.Chromatin remodeling, DNA damage repair and aging.

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P2860

Blocking protein farnesyltransferase improves nuclear shape in fibroblasts from humans with progeroid syndromes

http://www.wikidata.org/entity/Q33930224

description

2005 nî lūn-bûn

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2005 թուականի Օգոստոսին հրատարակուած գիտական յօդուած

@hyw

2005 թվականի օգոստոսին հրատարակված գիտական հոդված

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2005年の論文

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2005年論文

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2005年論文

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2005年論文

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2005年論文

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2005年論文

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Blocking protein farnesyltrans ...... umans with progeroid syndromes

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Blocking protein farnesyltrans ...... umans with progeroid syndromes

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Blocking protein farnesyltrans ...... umans with progeroid syndromes

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Blocking protein farnesyltrans ...... umans with progeroid syndromes

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Blocking protein farnesyltrans ...... umans with progeroid syndromes

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PNAS.0505767102

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Proceedings of the National Academy of Sciences of the United States of America

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Blocking protein farnesyltrans ...... umans with progeroid syndromes

@en

P2093

Anne P Beigneux

http://www.w3.org/2001/XMLSchema#string

Casey L Moulson

http://www.w3.org/2001/XMLSchema#string

Jeffrey H Miner

http://www.w3.org/2001/XMLSchema#string

Julia I Toth

http://www.w3.org/2001/XMLSchema#string

Loren G Fong

http://www.w3.org/2001/XMLSchema#string

Michael H Gelb

http://www.w3.org/2001/XMLSchema#string

Shao H Yang

http://www.w3.org/2001/XMLSchema#string

Xin Qiao

http://www.w3.org/2001/XMLSchema#string

P2860

Accumulation of mutant lamin A causes progressive changes in nuclear architecture in Hutchinson-Gilford progeria syndrome

Recurrent de novo point mutations in lamin A cause Hutchinson-Gilford progeria syndrome

LMNA mutations in atypical Werner's syndrome

Life at the edge: the nuclear envelope and human disease

Farnesyltransferase inhibitor tipifarnib is well tolerated, induces stabilization of disease, and inhibits farnesylation and oncogenic/tumor survival pathways in patients with advanced multiple myeloma

Heterozygosity for Lmna deficiency eliminates the progeria-like phenotypes in Zmpste24-deficient mice

Blocking protein farnesyltransferase improves nuclear blebbing in mouse fibroblasts with a targeted Hutchinson-Gilford progeria syndrome mutation.

Zmpste24 deficiency in mice causes spontaneous bone fractures, muscle weakness, and a prelamin A processing defect.

Homozygous and compound heterozygous mutations in ZMPSTE24 cause the laminopathy restrictive dermopathy.

Head and/or CaaX domain deletions of lamin proteins disrupt preformed lamin A and C but not lamin B structure in mammalian cells.

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12873-12878

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scholarly article

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10.1073/PNAS.0505767102

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P433

36

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102

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Stephen G. Young

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2005-08-29T00:00:00Z

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7630775

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16129834

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1193538

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