Mutations in human cardiac troponin I that are associated with restrictive cardiomyopathy affect basal ATPase activity and the calcium sensitivity of force development.
about
Gene regulation, alternative splicing, and posttranslational modification of troponin subunits in cardiac development and adaptation: a focused reviewCardiac troponin mutations and restrictive cardiomyopathyA troponin T mutation that causes infantile restrictive cardiomyopathy increases Ca2+ sensitivity of force development and impairs the inhibitory properties of troponinMolecular basis of calcium-sensitizing and desensitizing mutations of the human cardiac troponin C regulatory domain: a multi-scale simulation studyWhy Is there a Limit to the Changes in Myofilament Ca2+-Sensitivity Associated with Myopathy Causing Mutations?Top-down quantitative proteomics identified phosphorylation of cardiac troponin I as a candidate biomarker for chronic heart failureEffects of thin and thick filament proteins on calcium binding and exchange with cardiac troponin CMyosin transducer mutations differentially affect motor function, myofibril structure, and the performance of skeletal and cardiac musclesThe molecular effects of skeletal muscle myosin regulatory light chain phosphorylation.Diastolic dysfunction and thin filament dysregulation resulting from excitation-contraction uncoupling in a mouse model of restrictive cardiomyopathy.Correcting diastolic dysfunction by Ca2+ desensitizing troponin in a transgenic mouse model of restrictive cardiomyopathyPredicting cardiomyopathic phenotypes by altering Ca2+ affinity of cardiac troponin C.Molecular etiology of idiopathic cardiomyopathy.Disease-related cardiac troponins alter thin filament Ca2+ association and dissociation rates.Dual regulatory functions of the thin filament revealed by replacement of the troponin I inhibitory peptide with a linkerLow temperature dynamic mapping reveals unexpected order and disorder in troponin.Fetal cardiac troponin isoforms rescue the increased Ca2+ sensitivity produced by a novel double deletion in cardiac troponin T linked to restrictive cardiomyopathy: a clinical, genetic, and functional approachA cis-regulatory mutation in troponin-I of Drosophila reveals the importance of proper stoichiometry of structural proteins during muscle assembly.Genetics of inherited cardiomyopathy.Engineered troponin C constructs correct disease-related cardiac myofilament calcium sensitivity.Insights into restrictive cardiomyopathy from clinical and animal studies.Negative charges at protein kinase C sites of troponin I stabilize the inactive state of actinGenetic engineering and therapy for inherited and acquired cardiomyopathies.Allele and species dependent contractile defects by restrictive and hypertrophic cardiomyopathy-linked troponin I mutantsAnalysis of the molecular pathogenesis of cardiomyopathy-causing cTnT mutants I79N, ΔE96, and ΔK210.Role of cardiac troponin I carboxy terminal mobile domain and linker sequence in regulating cardiac contraction.The functional significance of the last 5 residues of the C-terminus of cardiac troponin I.Sarcomere neutralization in inherited cardiomyopathy: small-molecule proof-of-concept to correct hyper-Ca2+-sensitive myofilamentsRegulatory light chain mutations associated with cardiomyopathy affect myosin mechanics and kineticsSome cardiomyopathy-causing troponin I mutations stabilize a functional intermediate actin state.Designing heart performance by gene transfer.Diverse Phenotypic Expression of Cardiomyopathies in a Family with TNNI3 p.Arg145Trp Mutation.The myosin-activated thin filament regulatory state, M⁻-open: a link to hypertrophic cardiomyopathy (HCM).Experimental models of inherited cardiomyopathy and its therapeutics.Precision medicine approach to genetic cardiomyopathy.Proteasome dysfunction in cardiomyopathies.Restrictive Cardiomyopathy Caused by Troponin Mutations: Application of Disease Animal Models in Translational StudiesMesoscopic analysis of motion and conformation of cross-bridges.Diagnostic disparity and identification of two TNNI3 gene mutations, one novel and one arising de novo, in South African patients with restrictive cardiomyopathy and focal ventricular hypertrophy.Molecular mechanisms of sarcomere dysfunction in dilated and hypertrophic cardiomyopathy.
P2860
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P2860
Mutations in human cardiac troponin I that are associated with restrictive cardiomyopathy affect basal ATPase activity and the calcium sensitivity of force development.
description
2005 nî lūn-bûn
@nan
2005年の論文
@ja
2005年論文
@yue
2005年論文
@zh-hant
2005年論文
@zh-hk
2005年論文
@zh-mo
2005年論文
@zh-tw
2005年论文
@wuu
2005年论文
@zh
2005年论文
@zh-cn
name
Mutations in human cardiac tro ...... sitivity of force development.
@en
Mutations in human cardiac tro ...... sitivity of force development.
@nl
type
label
Mutations in human cardiac tro ...... sitivity of force development.
@en
Mutations in human cardiac tro ...... sitivity of force development.
@nl
prefLabel
Mutations in human cardiac tro ...... sitivity of force development.
@en
Mutations in human cardiac tro ...... sitivity of force development.
@nl
P2093
P2860
P356
P1476
Mutations in human cardiac tro ...... sitivity of force development.
@en
P2093
Aldrin V Gomes
James D Potter
Jingsheng Liang
P2860
P304
30909-30915
P356
10.1074/JBC.M500287200
P407
P577
2005-06-15T00:00:00Z