Lamin A/C haploinsufficiency causes dilated cardiomyopathy and apoptosis-triggered cardiac conduction system disease. - Wikidata - awgldk - TriplyDB

Lamin A/C haploinsufficiency causes dilated cardiomyopathy and apoptosis-triggered cardiac conduction system disease.

Lamin A/C haploinsufficiency causes dilated cardiomyopathy and apoptosis-triggered cardiac conduction system disease.

http://www.wikidata.org/entity/Q34446739

about

Lamin A/C haploinsufficiency modulates the differentiation potential of mouse embryonic stem cells Cardiomyocyte-specific expression of lamin a improves cardiac function in Lmna-/- mice Cardiac disease and arrhythmogenesis: Mechanistic insights from mouse models Genetic Variations Leading to Familial Dilated Cardiomyopathy Recent advances in animal and human pluripotent stem cell modeling of cardiac laminopathy The cardiac conduction system Laminopathies and the long strange trip from basic cell biology to therapy LMNA E82K mutation activates FAS and mitochondrial pathways of apoptosis in heart tissue specific transgenic mice Diseases of the nuclear envelope.Uncoordinated transcription and compromised muscle function in the lmna-null mouse model of Emery- Emery-Dreyfuss muscular dystrophy.Attenuated hypertrophic response to pressure overload in a lamin A/C haploinsufficiency mouse Deletion of the lmna gene induces growth delay and serum biochemical changes in C57BL/6 mice Direct synthesis of lamin A, bypassing prelamin a processing, causes misshapen nuclei in fibroblasts but no detectable pathology in mice.RNAi-based gene therapy for dominant Limb Girdle Muscular Dystrophies.Differential temporal and spatial progerin expression during closure of the ductus arteriosus in neonates Cardiac fibrosis in mice with hypertrophic cardiomyopathy is mediated by non-myocyte proliferation and requires Tgf-β.LINCing lamin B2 to neuronal migration: growing evidence for cell-specific roles of B-type lamins.Cellular mechanosensing: getting to the nucleus of it all.Investigating the purpose of prelamin A processing Post-natal myogenic and adipogenic developmental: defects and metabolic impairment upon loss of A-type lamins.Energetics, epigenetics, mitochondrial genetics.Nuclear lamins: key regulators of nuclear structure and activities Sustained accumulation of prelamin A and depletion of lamin A/C both cause oxidative stress and mitochondrial dysfunction but induce different cell fates Exome-wide association study reveals novel susceptibility genes to sporadic dilated cardiomyopathy.Modeling of lamin A/C mutation premature cardiac aging using patient‐specific induced pluripotent stem cells.Targeting Mitogen-Activated Protein Kinase Signaling in Mouse Models of Cardiomyopathy Caused by Lamin A/C Gene Mutations Deleterious assembly of the lamin A/C mutant p.S143P causes ER stress in familial dilated cardiomyopathy.Nuclear shape, mechanics, and mechanotransduction.Generation and characterization of a conditional deletion allele for Lmna in mice.Defective skeletal muscle growth in lamin A/C-deficient mice is rescued by loss of Lap2α.Molecular mechanisms of inherited arrhythmias.Genetic and ultrastructural studies in dilated cardiomyopathy patients: a large deletion in the lamin A/C gene is associated with cardiomyocyte nuclear envelope disruption.Animal models of arrhythmogenic cardiomyopathy.Role of nuclear Lamin A/C in cardiomyocyte functions.What Should the Cardiologist know about Lamin Disease?Murine Electrophysiological Models of Cardiac Arrhythmogenesis.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.Nesprin-1 mutations in human and murine cardiomyopathy.Modeling Treatment Response for Lamin A/C Related Dilated Cardiomyopathy in Human Induced Pluripotent Stem Cells.

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P2860

Lamin A/C haploinsufficiency causes dilated cardiomyopathy and apoptosis-triggered cardiac conduction system disease.

http://www.wikidata.org/entity/Q34446739

description

2007 nî lūn-bûn

@nan

2007 թուականի Դեկտեմբերին հրատարակուած գիտական յօդուած

@hyw

2007 թվականի դեկտեմբերին հրատարակված գիտական հոդված

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2007年の論文

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2007年論文

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2007年論文

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2007年論文

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2007年論文

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2007年論文

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2007年论文

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name

Lamin A/C haploinsufficiency c ...... iac conduction system disease.

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Lamin A/C haploinsufficiency c ...... iac conduction system disease.

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Lamin A/C haploinsufficiency c ...... iac conduction system disease.

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type

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Lamin A/C haploinsufficiency c ...... iac conduction system disease.

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Lamin A/C haploinsufficiency c ...... iac conduction system disease.

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Lamin A/C haploinsufficiency c ...... iac conduction system disease.

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prefLabel

Lamin A/C haploinsufficiency c ...... iac conduction system disease.

@ast

Lamin A/C haploinsufficiency c ...... iac conduction system disease.

@en

Lamin A/C haploinsufficiency c ...... iac conduction system disease.

@nl

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J.YJMCC.2007.11.008

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Journal of Molecular and Cellular Cardiology

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Lamin A/C haploinsufficiency c ...... diac conduction system disease

@en

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Charles I Berul

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Christine E Seidman

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Colin L Stewart

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Cordula M Wolf

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David A Conner

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Dorothy M Branco

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Hiroko Wakimoto

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J G Seidman

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Libin Wang

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Megan Sherwood

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

P2860

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

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

Missense mutations in the rod domain of the lamin A/C gene as causes of dilated cardiomyopathy and conduction-system disease

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

The T-Box transcription factor Tbx5 is required for the patterning and maturation of the murine cardiac conduction system

Bone morphogenetic protein-2 inhibits serum deprivation-induced apoptosis of neonatal cardiac myocytes through activation of the Smad1 pathway

Defects in nuclear structure and function promote dilated cardiomyopathy in lamin A/C-deficient mice

Activation of MAPK pathways links LMNA mutations to cardiomyopathy in Emery-Dreifuss muscular dystrophy.

Apoptosis as a possible cause of gradual development of complete heart block and fatal arrhythmias associated with absence of the AV node, sinus node, and internodal pathways.

Cardiac dysrhythmias,cardiomyopathy and muscular dystrophy in patients with Emery-Dreifuss muscular dystrophy and limb-girdle muscular dystrophy type 1B.

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293-303

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10.1016/J.YJMCC.2007.11.008

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2

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44

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Patrick G. Burgon

Glenn I Fishman

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2007-12-03T00:00:00Z

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5668555

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18182166

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haploinsufficiency

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3011813

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