A single autosomal dominant mutation in KCNN3 (guanine (mutant) substitution for cytosine (wild type) at nucleotide 1348 in coding DNA (or c.1348 C>G) and the KCa2.3 channel subunit (leucine (mutant) substitution for valine (wild type) at amino acid 450 (or V450L)) underlies development of idiopathic non-cirrhotic portal hypertension [119]. The gene discussed is KCNN3; the disease is liver disorder.