An accumulation of non-farnesylated prelamin A causes cardiomyopathy but not progeria.
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Targeting protein prenylation in progeriaBlocking protein farnesylation improves nuclear shape abnormalities in keratinocytes of mice expressing the prelamin A variant in Hutchinson-Gilford progeria syndromeMolecular insights into the premature aging disease progeriaWhen lamins go bad: nuclear structure and diseaseMouse models of laminopathiesCardiomyocyte-specific expression of lamin a improves cardiac function in Lmna-/- miceEmbryonic senescence and laminopathies in a progeroid zebrafish modelProtein farnesylation inhibitors cause donut-shaped cell nuclei attributable to a centrosome separation defectFarnesylation of lamin B1 is important for retention of nuclear chromatin during neuronal migration.HP1α mediates defective heterochromatin repair and accelerates senescence in Zmpste24-deficient cells.Direct synthesis of lamin A, bypassing prelamin a processing, causes misshapen nuclei in fibroblasts but no detectable pathology in mice.Nuclear lamins and neurobiology.Absence of progeria-like disease phenotypes in knock-in mice expressing a non-farnesylated version of progerin.Investigating the purpose of prelamin A processingMice that express farnesylated versions of prelamin A in neurons develop achalasia.iTRAQ-based analysis of progerin expression reveals mitochondrial dysfunction, reactive oxygen species accumulation and altered proteostasis.Understanding the roles of nuclear A- and B-type lamins in brain development.Inhibitors of protein geranylgeranyltransferase-I lead to prelamin A accumulation in cells by inhibiting ZMPSTE24Neonatal progeria: increased ratio of progerin to lamin A leads to progeria of the newbornNuclear lamins in the brain - new insights into function and regulation.Myopathic lamin mutations impair nuclear stability in cells and tissue and disrupt nucleo-cytoskeletal coupling.Targeting Mitogen-Activated Protein Kinase Signaling in Mouse Models of Cardiomyopathy Caused by Lamin A/C Gene MutationsLamin B1 and lamin B2 are long-lived proteins with distinct functions in retinal development.Prelamin A processing, accumulation and distribution in normal cells and laminopathy disorders.Investigation of splicing changes and post-translational processing of LMNA in sporadic inclusion body myositis.A High Throughput Phenotypic Screening reveals compounds that counteract premature osteogenic differentiation of HGPS iPS-derived mesenchymal stem cells.New Lmna knock-in mice provide a molecular mechanism for the 'segmental aging' in Hutchinson-Gilford progeria syndrome.Laminopathies: the molecular background of the disease and the prospects for its treatment.Lamins, laminopathies and disease mechanisms: possible role for proteasomal degradation of key regulatory proteins.Lamin A, farnesylation and agingSpeeding up the clock: The past, present and future of progeria.Current insights into LMNA cardiomyopathies: Existing models and missing LINCsA truncated lamin A in the Lmna -/- mouse line: implications for the understanding of laminopathies.Disruption of lamin B1 and lamin B2 processing and localization by farnesyltransferase inhibitors.Enhanced SRSF5 Protein Expression Reinforces Lamin A mRNA Production in HeLa Cells and Fibroblasts of Progeria Patients.Recent Advances in Understanding and Managing Cardiomyopathy.Structure and stability of the lamin A tail domain and HGPS mutant.Intermittent treatment with farnesyltransferase inhibitor and sulforaphane improves cellular homeostasis in Hutchinson-Gilford progeria fibroblasts.Prelamin A-mediated recruitment of SUN1 to the nuclear envelope directs nuclear positioning in human muscle.LMNA missense mutations causing familial partial lipodystrophy do not lead to an accumulation of prelamin A.
P2860
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P2860
An accumulation of non-farnesylated prelamin A causes cardiomyopathy but not progeria.
description
2010 nî lūn-bûn
@nan
2010 թուականի Ապրիլին հրատարակուած գիտական յօդուած
@hyw
2010 թվականի ապրիլին 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
An accumulation of non-farnesylated prelamin A causes cardiomyopathy but not progeria.
@ast
An accumulation of non-farnesylated prelamin A causes cardiomyopathy but not progeria.
@en
type
label
An accumulation of non-farnesylated prelamin A causes cardiomyopathy but not progeria.
@ast
An accumulation of non-farnesylated prelamin A causes cardiomyopathy but not progeria.
@en
prefLabel
An accumulation of non-farnesylated prelamin A causes cardiomyopathy but not progeria.
@ast
An accumulation of non-farnesylated prelamin A causes cardiomyopathy but not progeria.
@en
P2093
P2860
P356
P1476
An accumulation of non-farnesylated prelamin A causes cardiomyopathy but not progeria.
@en
P2093
Brandon S J Davies
Douglas A Andres
H Peter Spielmann
Jan Lammerding
Loren G Fong
Richard H Barnes
Shuxun Ren
P2860
P304
P356
10.1093/HMG/DDQ158
P577
2010-04-26T00:00:00Z