MiRP2 forms potassium channels in skeletal muscle with Kv3.4 and is associated with periodic paralysis
MiRP2 forms potassium channels in skeletal muscle with Kv3.4 and is associated with periodic paralysis
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DOI：
10.1016/s0092-8674(01)00207-0
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发表时间：
2001-01-26
期刊：
影响因子：
64.5
通讯作者：
Goldstein, SAN
中科院分区：
文献类型：
--
作者：
Abbott, GW;Butler, MH;Goldstein, SAN
骨骼肌的阈下电压门控钾通道含有MinK相关肽2（MiRP 2）和成孔亚基Kv3.4。MiRP 2-Kv3.4通道与Kv3.4通道的不同之处在于单位电导、电压依赖性激活、失活恢复、稳态开放概率和被肽毒素阻断。因此，MiRP 2-Kv3.4通道设定静息膜电位（RMP），并且不产生后超极化或累积失活以限制动作电位频率。在两个周期性麻痹家族的MiRP 2（KCNE 3）基因中发现了一个错义突变，并发现与疾病分离。突变MiRP 2-Kv3.4复合物表现出降低的电流密度和降低的设定RMP的能力。因此，MiRP 2与经典的钾通道亚基一起工作以控制骨骼肌功能和病理生理学。
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 mis-sense 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.