NOS1AP polymorphisms reduce NOS1 activity and interact with prolonged repolarization in arrhythmogenesis | Zendy

Carlotta Ronchi | Zendy; Joyce Bernardi | Zendy; Manuela Mura | Zendy; Manuela Stefanello | Zendy; Beatrice Badone | Zendy; Marcella Rocchetti | Zendy; Lia Crotti | Zendy; Paul A. Brink | Zendy; Peter J. Schwartz | Zendy; Massimiliano Gnecchi | Zendy; Antonio Zaza | Zendy

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NOS1AP polymorphisms reduce NOS1 activity and interact with prolonged repolarization in arrhythmogenesis

Author(s) -

Carlotta Ronchi,

Joyce Bernardi,

Manuela Mura,

Manuela Stefanello,

Beatrice Badone,

Marcella Rocchetti,

Lia Crotti,

Paul A. Brink,

Peter J. Schwartz,

Massimiliano Gnecchi,

Antonio Zaza

Publication year - 2020

Publication title -

cardiovascular research

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 2.774

H-Index - 219

eISSN - 1755-3245

pISSN - 0008-6363

DOI - 10.1093/cvr/cvaa036

Subject(s) - nos1 , biology , qt interval , repolarization , afterdepolarization , medicine , electrophysiology , neuroscience , endocrinology , nitric oxide synthase , nitric oxide

NOS1AP single-nucleotide polymorphisms (SNPs) correlate with QT prolongation and cardiac sudden death in patients affected by long QT syndrome type 1 (LQT1). NOS1AP targets NOS1 to intracellular effectors. We hypothesize that NOS1AP SNPs cause NOS1 dysfunction and this may converge with prolonged action-potential duration (APD) to facilitate arrhythmias. Here we test (i) the effects of NOS1 inhibition and their interaction with prolonged APD in a guinea pig cardiomyocyte (GP-CMs) LQT1 model; (ii) whether pluripotent stem cell-derived cardiomyocytes (hiPSC-CMs) from LQT1 patients differing for NOS1AP variants and mutation penetrance display a phenotype compatible with NOS1 deficiency.

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