Modulation of cardiac contractility by the phopholamban/SERCA2a regulatome

EG Kranias, RJ Hajjar - Circulation research, 2012 - Am Heart Assoc
Heart disease remains the leading cause of death and disability in the Western world.
Current therapies aim at treating the symptoms rather than the subcellular mechanisms …

Altered sarcoplasmic reticulum calcium cycling—targets for heart failure therapy

C Kho, A Lee, RJ Hajjar - Nature Reviews Cardiology, 2012 - nature.com
Cardiac myocyte function is dependent on the synchronized movements of Ca2+ into and
out of the cell, as well as between the cytosol and sarcoplasmic reticulum. These …

Phospholamban R14del mutation in patients diagnosed with dilated cardiomyopathy or arrhythmogenic right ventricular cardiomyopathy: evidence supporting the …

PA Van Der Zwaag, IAW Van Rijsingen… - European journal of …, 2012 - Wiley Online Library
Aims To investigate whether phospholamban gene (PLN) mutations underlie patients
diagnosed with either arrhythmogenic right ventricular cardiomyopathy (ARVC) or idiopathic …

[PDF][PDF] Cantu syndrome is caused by mutations in ABCC9

BWM Van Bon, C Gilissen, DK Grange… - The American Journal of …, 2012 - cell.com
Cantú syndrome is a rare disorder characterized by congenital hypertrichosis, neonatal
macrosomia, a distinct osteochondrodysplasia, and cardiomegaly. Using an exome …

Remodeling of calcium handling in human heart failure

Q Lou, A Janardhan, IR Efimov - Calcium Signaling, 2012 - Springer
Heart failure (HF) is an increasing public health problem accelerated by a rapidly aging
global population. Despite considerable progress in managing the disease, the …

Anchoring proteins as regulators of signaling pathways

A Perino, A Ghigo, JD Scott, E Hirsch - Circulation research, 2012 - Am Heart Assoc
Spatial and temporal organization of signal transduction is coordinated through the
segregation of signaling enzymes in selected cellular compartments. This highly evolved …

[HTML][HTML] Hydrophobic imbalance in the cytoplasmic domain of phospholamban is a determinant for lethal dilated cardiomyopathy

DK Ceholski, CA Trieber, HS Young - Journal of Biological Chemistry, 2012 - ASBMB
The sarco (endo) plasmic reticulum calcium ATPase (SERCA) and its regulatory partner
phospholamban (PLN) are essential for myocardial contractility. Arg 9→ Cys (R9C) and Arg …

[HTML][HTML] Lethal, hereditary mutants of phospholamban elude phosphorylation by protein kinase A

DK Ceholski, CA Trieber, CFB Holmes… - Journal of Biological …, 2012 - ASBMB
The sarcoplasmic reticulum calcium pump (SERCA) and its regulator, phospholamban, are
essential components of cardiac contractility. Phospholamban modulates contractility by …

Cardiomyopathy classification: ongoing debate in the genomics era

C McCartan, R Mason, SR Jayasinghe… - Biochemistry …, 2012 - Wiley Online Library
Cardiomyopathies represent a group of diseases of the myocardium of the heart and include
diseases both primarily of the cardiac muscle and systemic diseases leading to adverse …

Mice deleted for heart-type cytochrome c oxidase subunit 7a1 develop dilated cardiomyopathy

M Hüttemann, S Klewer, I Lee, A Pecinova, P Pecina… - Mitochondrion, 2012 - Elsevier
Subunit 7a of mouse cytochrome c oxidase (Cox) displays a contractile muscle-specific
isoform, Cox7a1, that is the major cardiac form. To gain insight into the role of this isoform …