title: Andersen syndrome: the newest variant of the hereditary-familial long QT syndrome.
abstract: 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.
authors.affiliation: ABC University Fundação Santo André, São Paulo, Brazil. riera@uol.com.br
authors.forename: Andrés
authors.initials: A
authors.lastname: Ricardo Pérez Riera
authors.forename: Celso
authors.initials: C
authors.lastname: Ferreira
authors.forename: Sérgio J
authors.initials: SJ
authors.lastname: Dubner
authors.forename: Edgardo
authors.initials: E
authors.lastname: Schapachnik
mesh_terms: D000200:Action Potentials / Q000235:genetics; D002257:Carbonic Anhydrase Inhibitors / Q000627:therapeutic use; D004562:Electrocardiography; D020022:Genetic Predisposition to Disease / Q000209:etiology / Q000235:genetics; D006011:Glycogen Storage Disease Type IV / Q000188:drug therapy / Q000209:etiology / Q000235:genetics*; D006329:Heart Conduction System / Q000473:pathology / Q000503:physiopathology; D006801:Humans; D008133:Long QT Syndrome / Q000188:drug therapy / Q000209:etiology / Q000235:genetics*; D010245:Paralyses, Familial Periodic / Q000188:drug therapy / Q000209:etiology / Q000235:genetics; D015221:Potassium Channels / Q000235:genetics / Q000378:metabolism
chemical_list: D002257:Carbonic Anhydrase Inhibitors; D015221:Potassium Channels