Potassium channel subunits encoded by the KCNE gene family: physiology and pathophysiology of the MinK-related peptides (MiRPs).
Voltage-gated potassium channels provide tightly Controlled, ion-specific pathways across membranes and are key to the normal function of nerves muscles. They arise from the assembly of four pore-forming proteins called alpha-subunits. To attain the properties of native currents, alpha-subunits interact with additional molecules such as the mink-related peptides (MiRPs), single-transmembrane subunits encoded by the KCNE genes. Significantly, mutations in KCNE 1, 2 and 3 have been linked either to life-threatening cardiac arrhythmia or a disorder of skeletal muscle, familial periodic paralysis. The capacity of MiRPs to partner with multiple alpha-subunits in experimental cells appears to reflect still undiscovered roles for the KCNE-encoded peptides in vivo. Here, we consider these unique peptides in health disease and discuss future research directions.
Abbott
G W
GW
Department of Pediatrics, Boyer Center for Molecular Medicine, Yale University School of Medicine, New Haven, CT 06536, USA.
Goldstein
S A
SA
D000595:Amino Acid Sequence; D000818:Animals; D001145:Arrhythmias, Cardiac / Q000235:genetics; D027682:Cation Transport Proteins / Q000378:metabolism; D004553:Electric Conductivity; D051638:Ether-A-Go-Go Potassium Channels; D005544:Forecasting; D006801:Humans; D015640:Ion Channel Gating; D008954:Models, Biological; D008969:Molecular Sequence Data; D020125:Mutation, Missense; D015221:Potassium Channels / Q000737:chemistry / Q000235:genetics* / Q000378:metabolism / Q000502:physiology*; D024642:Potassium Channels, Voltage-Gated; D017434:Protein Structure, Tertiary; D021122:Protein Subunits / Q000235:genetics* / Q000378:metabolism; D017386:Sequence Homology, Amino Acid
D027682:Cation Transport Proteins; D051638:Ether-A-Go-Go Potassium Channels; C092509:KCNH6 protein, human; D015221:Potassium Channels; D024642:Potassium Channels, Voltage-Gated; D021122:Protein Subunits; C059983:potassium channel protein I(sk)