Conditional expression of mutant M-line titins results in cardiomyopathy with altered sarcomere structure.
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Obscurin targets ankyrin-B and protein phosphatase 2A to the cardiac M-lineRequirement for serum response factor for skeletal muscle growth and maturation revealed by tissue-specific gene deletion in miceMyosin accumulation and striated muscle myopathy result from the loss of muscle RING finger 1 and 3nfi-I affects behavior and life-span in C. elegans but is not essential for DNA replication or survival.Titin-based tension in the cardiac sarcomere: molecular origin and physiological adaptationsEvidence for the mechanosensor function of filamin in tissue development.Normal sorting but defective endocytosis of the low density lipoprotein receptor in mice with autosomal recessive hypercholesterolemiaTargeted deletion of titin N2B region leads to diastolic dysfunction and cardiac atrophyM line-deficient titin causes cardiac lethality through impaired maturation of the sarcomereThe ATPase-dependent chaperoning activity of Hsp90a regulates thick filament formation and integration during skeletal muscle myofibrillogenesisGenetic epidemiology of titin-truncating variants in the etiology of dilated cardiomyopathy.Depletion of zebrafish Tcap leads to muscular dystrophy via disrupting sarcomere-membrane interaction, not sarcomere assemblyDOT1L regulates dystrophin expression and is critical for cardiac function.Titin-based mechanosensing and signaling: role in diaphragm atrophy during unloading?Cardiac titin and heart disease.Muscle giants: molecular scaffolds in sarcomerogenesis.Caenorhabditis elegans UNC-96 is a new component of M-lines that interacts with UNC-98 and paramyosin and is required in adult muscle for assembly and/or maintenance of thick filaments.Titin visualization in real time reveals an unexpected level of mobility within and between sarcomeresLoss of muscle-specific RING-finger 3 predisposes the heart to cardiac rupture after myocardial infarction.Altered contractility of skeletal muscle in mice deficient in titin's M-band region.Pressure Overload by Transverse Aortic Constriction Induces Maladaptive Hypertrophy in a Titin-Truncated Mouse ModelTitin is a major human disease gene.Single-molecule force spectroscopy reveals a stepwise unfolding of Caenorhabditis elegans giant protein kinase domains.The tight junction protein CAR regulates cardiac conduction and cell-cell communication.Mechanisms of muscle gene regulation in the electric organ of Sternopygus macrurus.Assembly and maintenance of the sarcomere night and day.Roles and potential therapeutic targets of the ubiquitin proteasome system in muscle wasting.Alternative Splicing, Internal Promoter, Nonsense-Mediated Decay, or All Three: Explaining the Distribution of Truncation Variants in Titin.Tropomodulin1 is required in the heart but not the yolk sac for mouse embryonic development.Increasing Role of Titin Mutations in Neuromuscular Disorders.Cardiac titin: a multifunctional giantAssembly and dynamics of myofibrils.Skeletal muscle, autophagy, and physical activity: the ménage à trois of metabolic regulation in health and disease.A rising titan: TTN review and mutation update.Stomach-specific calpain, nCL-2, localizes in mucus cells and proteolyzes the beta-subunit of coatomer complex, beta-COP.Titin mutations: the fall of Goliath.Functional analysis of slow myosin heavy chain 1 and myomesin-3 in sarcomere organization in zebrafish embryonic slow muscles.Animal Models of Congenital Cardiomyopathies Associated With Mutations in Z-Line Proteins.Titin and Nebulin in Thick and Thin Filament Length Regulation.Mechanically induced titin kinase activation studied by force-probe molecular dynamics simulations.
P2860
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P2860
Conditional expression of mutant M-line titins results in cardiomyopathy with altered sarcomere structure.
description
2002 nî lūn-bûn
@nan
2002年の論文
@ja
2002年学术文章
@wuu
2002年学术文章
@zh-cn
2002年学术文章
@zh-hans
2002年学术文章
@zh-my
2002年学术文章
@zh-sg
2002年學術文章
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2002年學術文章
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2002年學術文章
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name
Conditional expression of muta ...... h altered sarcomere structure.
@en
Conditional expression of muta ...... h altered sarcomere structure.
@nl
type
label
Conditional expression of muta ...... h altered sarcomere structure.
@en
Conditional expression of muta ...... h altered sarcomere structure.
@nl
prefLabel
Conditional expression of muta ...... h altered sarcomere structure.
@en
Conditional expression of muta ...... h altered sarcomere structure.
@nl
P2093
P2860
P356
P1476
Conditional expression of muta ...... h altered sarcomere structure.
@en
P2093
Christian C Witt
Henk Granzier
James A Richardson
Joachim Herz
Mark McNabb
Norbert Hübner
Robert E Hammer
Siegfried Labeit
P2860
P304
P356
10.1074/JBC.M211723200
P407
P577
2002-12-02T00:00:00Z