Mutation E169K in junctophilin-2 causes atrial fibrillation due to impaired RyR2 stabilization

DL Beavers, W Wang, S Ather, N Voigt… - Journal of the American …, 2013 - jacc.org
Objectives: This study sought to study the role of junctophilin-2 (JPH2) in atrial fibrillation
(AF). Background: JPH2 is believed to have an important role in sarcoplasmic reticulum (SR) …

[HTML][HTML] Mutation of junctophilin type 2 associated with hypertrophic cardiomyopathy

Y Matsushita, T Furukawa, H Kasanuki… - Journal of human …, 2007 - nature.com
Junctophilin subtypes, designated as JPH1∼ 4, are protein components of junctional
complexes and play essential roles in cellular Ca 2+ signaling in excitable cells. Knockout …

Junctophilin-2 expression silencing causes cardiocyte hypertrophy and abnormal intracellular calcium-handling

AP Landstrom, CA Kellen, SS Dixit… - Circulation: Heart …, 2011 - Am Heart Assoc
Background—Junctophilin-2 (JPH2), a protein expressed in the junctional membrane
complex, is necessary for proper intracellular calcium (Ca2+) signaling in cardiac myocytes …

Mutations in JPH2-encoded junctophilin-2 associated with hypertrophic cardiomyopathy in humans

AP Landstrom, N Weisleder, KB Batalden… - Journal of molecular and …, 2007 - Elsevier
Junctophilin-2 (JPH2) is a cardiac specific member of the junctophilins, a newly
characterized family of junctional membrane complex proteins important in physically …

Emerging roles of junctophilin-2 in the heart and implications for cardiac diseases

DL Beavers, AP Landstrom, DY Chiang… - Cardiovascular …, 2014 - academic.oup.com
Cardiomyocytes rely on a highly specialized subcellular architecture to maintain normal
cardiac function. In a little over a decade, junctophilin-2 (JPH2) has become recognized as a …

Disrupted junctional membrane complexes and hyperactive ryanodine receptors after acute junctophilin knockdown in mice

RJ Van Oort, A Garbino, W Wang, SS Dixit… - Circulation, 2011 - Am Heart Assoc
Background—Excitation-contraction coupling in striated muscle requires proper
communication of plasmalemmal voltage-activated Ca2+ channels and Ca2+ release …

[HTML][HTML] Emerging role of junctophilin-2 as a regulator of calcium handling in the heart

A Garbino, XHT Wehrens - Acta Pharmacologica Sinica, 2010 - nature.com
Abstract Junctophilin-2 (JPH2) is a membrane-binding protein that plays a key role in the
organization of the junctional membrane complex (JMC) in cardiac myocytes. JPH2 is …

Inhibition of CaMKII phosphorylation of RyR2 prevents induction of atrial fibrillation in FKBP12. 6 knockout mice

N Li, T Wang, W Wang, MJ Cutler, Q Wang… - Circulation …, 2012 - Am Heart Assoc
Rationale: Abnormal calcium release from sarcoplasmic reticulum (SR) is considered an
important trigger of atrial fibrillation (AF). Whereas increased Ca2+/calmodulin-dependent …

Reduced junctional Na+/Ca2+-exchanger activity contributes to sarcoplasmic reticulum Ca2+ leak in junctophilin-2-deficient mice

W Wang, AP Landstrom, Q Wang… - American Journal …, 2014 - journals.physiology.org
Expression silencing of junctophilin-2 (JPH2) in mouse heart leads to ryanodine receptor
type 2 (RyR2)-mediated sarcoplasmic reticulum (SR) Ca2+ leak and rapid development of …

The selective RyR2 inhibitor ent-verticilide suppresses atrial fibrillation susceptibility caused by Pitx2 deficiency

K Kim, DJ Blackwell, SL Yuen, MP Thorpe… - Journal of molecular and …, 2023 - Elsevier
Atrial fibrillation (AF) is the most common sustained cardiac arrhythmia and a major cause of
stroke and morbidity. The strongest genetic risk factors for AF in humans are variants on …