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.
Department of Pediatrics, Boyer Center for Molecular Medicine, Yale University School of Medicine, New Haven, CT 06536, USA.
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GW
Abbott
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Goldstein
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)