An accumulation of non-farnesylated prelamin A causes cardiomyopathy but not progeria. - Wikidata - wikimedia - TriplyDB

An accumulation of non-farnesylated prelamin A causes cardiomyopathy but not progeria.

An accumulation of non-farnesylated prelamin A causes cardiomyopathy but not progeria.

http://www.wikidata.org/entity/Q33904465

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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 Molecular insights into the premature aging disease progeria When lamins go bad: nuclear structure and disease Mouse models of laminopathies Cardiomyocyte-specific expression of lamin a improves cardiac function in Lmna-/- mice Embryonic senescence and laminopathies in a progeroid zebrafish model Protein farnesylation inhibitors cause donut-shaped cell nuclei attributable to a centrosome separation defect Farnesylation 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 processing Mice 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 ZMPSTE24 Neonatal progeria: increased ratio of progerin to lamin A leads to progeria of the newborn Nuclear 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 Mutations Lamin 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 aging Speeding up the clock: The past, present and future of progeria.Current insights into LMNA cardiomyopathies: Existing models and missing LINCs A 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.

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P2860

An accumulation of non-farnesylated prelamin A causes cardiomyopathy but not progeria.

http://www.wikidata.org/entity/Q33904465

description

2010 nî lūn-bûn

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2010 թուականի Ապրիլին հրատարակուած գիտական յօդուած

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2010 թվականի ապրիլին հրատարակված գիտական հոդված

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2010年の論文

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2010年論文

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2010年論文

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2010年論文

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2010年論文

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2010年論文

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2010年论文

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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

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Human Molecular Genetics

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An accumulation of non-farnesylated prelamin A causes cardiomyopathy but not progeria.

@en

P2093

Brandon S J Davies

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

Douglas A Andres

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

H Peter Spielmann

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Jan Lammerding

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Loren G Fong

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Richard H Barnes

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Shuxun Ren

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Yiping Tu

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P2860

Mutations in the gene encoding lamin A/C cause autosomal dominant Emery-Dreifuss muscular dystrophy

Crystallographic analysis of CaaX prenyltransferases complexed with substrates defines rules of protein substrate selectivity

Inhibiting farnesylation of progerin prevents the characteristic nuclear blebbing of Hutchinson-Gilford progeria syndrome

Loss of A-type lamin expression compromises nuclear envelope integrity leading to muscular dystrophy

Identification of mutations in the gene encoding lamins A/C in autosomal dominant limb girdle muscular dystrophy with atrioventricular conduction disturbances (LGMD1B)

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

The processing pathway of prelamin A

Protein prenyltransferases

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

Protein farnesyltransferase in embryogenesis, adult homeostasis, and tumor development

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10.1093/HMG/DDQ158

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13

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19

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Stephen G. Young

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2010-04-26T00:00:00Z

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43345571

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20421363

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2883346

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