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Inward-rectifying potassium channelopathies: new insights into disorders of sodium and potassium homeostasis

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Abstract

Inward-rectifying potassium (Kir) channels allow more inward than outward potassium flux when channels are open in mammalian cells. At physiological resting membrane potentials, however, they predominantly mediate outward potassium flux and play important roles in regulating the resting membrane potential in diverse cell types and potassium secretion in the kidneys. Mutations of Kir channels cause human hereditary diseases collectively called Kir channelopathies, many of which are characterized by disorders of sodium and potassium homeostasis. Studies on these genetic Kir channelopathies have shed light on novel pathophysiological mechanisms, including renal sodium and potassium handling, potassium shifting in skeletal muscles, and aldosterone production in the adrenal glands. Here, we review several recent advances in Kir channels and their clinical implications in sodium and potassium homeostasis.

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Acknowledgments

We thank Dr. Tom Chou in Providence St. Vincent Medical Center, Portland, Oregon, USA, for editing this manuscript. This work was supported by grants from NSC and NHRI of Taiwan (NSC102-2314-B-016-001, NHRI-EX103-10323SC to CJC).

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Authors and Affiliations

  1. Department of Medicine, Division of Nephrology, Tri-Service General Hospital, National Defense Medical Center, No. 325, Section 2, Cheng-Kung Road, Neihu 114, Taipei, Taiwan

    Chih-Jen Cheng, Chih-Chien Sung & Shih-Hua Lin

  2. Department of Medicine, Division of Nephrology, University of Texas Southwestern Medical Center, Dallas, TX, 75390-8856, USA

    Chou-Long Huang

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  1. Chih-Jen Cheng
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Cheng, CJ., Sung, CC., Huang, CL. et al. Inward-rectifying potassium channelopathies: new insights into disorders of sodium and potassium homeostasis. Pediatr Nephrol 30, 373–383 (2015). https://doi.org/10.1007/s00467-014-2764-0

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  • DOI: https://doi.org/10.1007/s00467-014-2764-0

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