Open AccessAndersen Syndrome: The Newest Variant of the Hereditary‐Familial Long QT Syndrome
Author(s) -
Ricardo Pérez Riera Andrés,
Ferreira Celso,
Dubner Sérgio J.,
Schapachnik Edgardo
Publication year - 2004
Publication title -
annals of noninvasive electrocardiology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.494
H-Index - 48
eISSN - 1542-474X
pISSN - 1082-720X
DOI - 10.1111/j.1542-474x.2004.92552.x
Subject(s) - channelopathy , medicine , short qt syndrome , brugada syndrome , inward rectifier potassium ion channel , potassium channel , long qt syndrome , ion channel , endocrinology , periodic paralysis , genetics , cardiology , qt interval , surgery , biology , receptor , paralysis
Andersen's Syndrome is a rare disease, hereditary with autosomal dominant transmission, of the ion channels of the sarcolemmal membranes of the cardiac and skeletal muscles (channelopathy), which affects chromosome 17 of the KCNJ2 gene, responsible for encoding the outward potassium delayed rectifier current KIR2.1, resulting in a loss or suppression of the function of this channel.