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Cell. January 26, 2001; 104 (2): 217-31.

MiRP2 forms potassium channels in skeletal muscle with Kv3.4 and is associated with periodic paralysis.

Abbott GW , Butler MH , Bendahhou S , Dalakas MC , Ptacek LJ , Goldstein SA .

The subthreshold, voltage-gated potassium channel of skeletal muscle is shown to contain MinK-related peptide 2 (MiRP2) and the pore-forming subunit Kv3.4. MiRP2-Kv3.4 channels differ from Kv3.4 channels in unitary conductance, voltage-dependent activation, recovery from inactivation, steady-state open probability, and block by a peptide toxin. Thus, MiRP2-Kv3.4 channels set resting membrane potential (RMP) and do not produce afterhyperpolarization or cumulative inactivation to limit action potential frequency. A missense mutation is identified in the gene for MiRP2 (KCNE3) in two families with periodic paralysis and found to segregate with the disease. Mutant MiRP2-Kv3.4 complexes exhibit reduced current density and diminished capacity to set RMP. Thus, MiRP2 operates with a classical potassium channel subunit to govern skeletal muscle function and pathophysiology.

PubMed ID: 11207363
Article link: Cell.
Grant support: MO1-RR00064 NCRR NIH HHS

Genes referenced: kcnc4 kcne1 kcne3 mink1

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