Molecular, subcellular, and arrhythmogenic mechanisms in genetic RyR2 disease

ED Fowler, S Zissimopoulos - Biomolecules, 2022 - mdpi.com
The ryanodine receptor (RyR2) has a critical role in controlling Ca2+ release from the
sarcoplasmic reticulum (SR) throughout the cardiac cycle. RyR2 protein has multiple …

Therapeutic approaches of ryanodine receptor-associated heart diseases

N Szentandrássy, ZÉ Magyar, J Hevesi… - International Journal of …, 2022 - mdpi.com
Cardiac diseases are the leading causes of death, with a growing number of cases
worldwide, posing a challenge for both healthcare and research. Therefore, the most …

“Ryanopathies” and RyR2 dysfunctions: can we further decipher them using in vitro human disease models?

Y Sleiman, A Lacampagne, AC Meli - Cell Death & Disease, 2021 - nature.com
The regulation of intracellular calcium (Ca2+) homeostasis is fundamental to maintain
normal functions in many cell types. The ryanodine receptor (RyR), the largest intracellular …

Is ryanodine receptor phosphorylation key to the fight or flight response and heart failure?

T Eschenhagen - The Journal of clinical investigation, 2010 - Am Soc Clin Investig
In situations of stress the heart beats faster and stronger. According to Marks and
colleagues, this response is, to a large extent, the consequence of facilitated Ca2+ release …

Mapping the ryanodine receptor FK506-binding protein subunit using fluorescence resonance energy transfer

RL Cornea, FR Nitu, M Samso, DD Thomas… - Journal of Biological …, 2010 - ASBMB
The 12-kDa FK506-binding proteins (FKBP12 and FKBP12. 6) are regulatory subunits of
ryanodine receptor (RyR) Ca 2+ release channels. To investigate the structural basis of …

Association of FK506 binding proteins with RyR channels–effect of CLIC2 binding on sub-conductance opening and FKBP binding

SJ Richardson, GA Steele, EM Gallant… - Journal of cell …, 2017 - journals.biologists.com
ABSTRACT Ryanodine receptor (RyR) Ca2+ channels are central to striated muscle
function and influence signalling in neurons and other cell types. Beneficially low RyR …

[HTML][HTML] Ryanodine receptor mutations in arrhythmia: the continuing mystery of channel dysfunction

NL Thomas, C Maxwell, S Mukherjee, AJ Williams - Febs Letters, 2010 - Elsevier
Mutations in RyR2 are causative of an inherited disorder which often results in sudden
cardiac death. Dysfunctional channel behaviour has been the subject of many investigations …

Identification of an amino-terminus determinant critical for ryanodine receptor/Ca2+ release channel function

M Seidel, CR de Meritens, L Johnson… - Cardiovascular …, 2021 - academic.oup.com
Aims The cardiac ryanodine receptor (RyR2), which mediates intracellular Ca2+ release to
trigger cardiomyocyte contraction, participates in development of acquired and inherited …

N-terminus oligomerization is conserved in intracellular calcium release channels

S Zissimopoulos, J Marsh, L Stannard… - Biochemical …, 2014 - portlandpress.com
Oligomerization of all three mammalian ryanodine receptor isoforms, a structural
requirement for normal intracellular Ca2+ release channel function, is displayed by the …

[PDF][PDF] Molecular, Subcellular, and Arrhythmogenic Mechanisms in Genetic RyR2 Disease. Biomolecules 2022, 12, 1030

ED Fowler, S Zissimopoulos - Molecular Pathogenesis of Cardiac …, 2022 - mdpi.com
The ryanodine receptor (RyR2) has a critical role in controlling Ca2+ release from the
sarcoplasmic reticulum (SR) throughout the cardiac cycle. RyR2 protein has multiple …