Dataset Information

Molecular Defects in Cardiac Myofilament Ca²⁺-Regulation Due to Cardiomyopathy-Linked Mutations Can Be Reversed by Small Molecules Binding to Troponin.

ABSTRACT: The inherited cardiomyopathies, hypertrophic cardiomyopathy (HCM) and dilated cardiomyopathy (DCM) are relatively common, potentially life-threatening and currently untreatable. Mutations are often in the contractile proteins of cardiac muscle and cause abnormal Ca²⁺ regulation via troponin. HCM is usually linked to higher myofilament Ca²⁺-sensitivity whilst in both HCM and DCM mutant tissue there is often an uncoupling of the relationship between troponin I (TnI) phosphorylation by PKA and modulation of myofilament Ca²⁺-sensitivity, essential for normal responses to adrenaline. The adrenergic response is blunted, and this may predispose the heart to failure under stress. At present there are no compounds or interventions that can prevent or treat sa

SUBMITTER: Sheehan A

PROVIDER: S-EPMC5881522 | biostudies-literature | 2018

REPOSITORIES: biostudies-literature

ACCESS DATA

Dataset Information

Molecular Defects in Cardiac Myofilament Ca²⁺-Regulation Due to Cardiomyopathy-Linked Mutations Can Be Reversed by Small Molecules Binding to Troponin.

OmicsDI is part of the ELIXIR infrastructure

Tweets

Dataset Information

Molecular Defects in Cardiac Myofilament Ca2+-Regulation Due to Cardiomyopathy-Linked Mutations Can Be Reversed by Small Molecules Binding to Troponin.

OmicsDI is part of the ELIXIR infrastructure

Tweets

Molecular Defects in Cardiac Myofilament Ca²⁺-Regulation Due to Cardiomyopathy-Linked Mutations Can Be Reversed by Small Molecules Binding to Troponin.