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ROMK and Bartter Syndrome Type 2

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Ion Channels and Transporters of Epithelia in Health and Disease

Part of the book series: Physiology in Health and Disease ((PIHD))

Abstract

The renal outer medullary potassium (K) channel, ROMK (aka Kir1.1, encoded by the KCNJ1 gene), is the founding member of the inwardly rectifying potassium channel family. It is primarily expressed in the kidney where it transports potassium into the pro-urine, important for maintaining salt reabsorption in the thick ascending limb, and potassium-homeostasis. Loss-of-function mutations in ROMK cause Bartter syndrome (type 2), a familial salt-losing nephropathy. This review provides a bedside-to-bench understanding of the disease, tracking the clinical phenotype to defects in tubule transport to the mutations in the channel.

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Authors and Affiliations

  1. Department of Physiology, University of Maryland School of Medicine, 655 West Baltimore St., BRB 5-029, Baltimore, MD, 21201, USA

    Paul A. Welling

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Correspondence to Paul A. Welling .

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  1. Department of Physiology, University of Otago , Dunedin, Otago, New Zealand

    Kirk L. Hamilton

  2. Department of Cell Biology, University of Pittsburgh, Pittsburgh, Pennsylvania, USA

    Daniel C Devor

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Welling, P.A. (2016). ROMK and Bartter Syndrome Type 2. In: Hamilton, K., Devor, D. (eds) Ion Channels and Transporters of Epithelia in Health and Disease. Physiology in Health and Disease. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-3366-2_19

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