Dominant-negative mutations in the KCNJ2 gene, encoding the K+ channel Kir2.1, also cause primary HypoPP in Andersen-Tawil syndrome, a disorder clinically distinct from HypoPP1 and 2 since the neuromuscular phenotype is associated with cardiac arrhythmia and bone deformities (Plaster et al., 2001). This evidence concerns the gene KCNJ2 and chronic obstructive pulmonary disease.