Mutant Cullin 3 causes familial hyperkalemic hypertension via dominant effects.

No abstract is available for this paper.

• Publication year

  2017

• Venue

  JCI Insight

• Publication date

  2017-12-21

• Fields of study

  Medicine, Chemistry

• Identifiers
  DOI 10.1172/jci.insight.96700PMID 29263298PMCID PMC5752287
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• No claims are published for this paper.

• No concepts are published for this paper.

• Constitutively Active SPAK Causes Hyperkalemia by Activating NCC and Remodeling Distal Tubules.

  2017influential reference
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• SPAK and OSR1 play essential roles in potassium homeostasis through actions on the distal convoluted tubule

  2016cited by this paper
• The CUL3/KLHL3-WNK-SPAK/OSR1 pathway as a target for antihypertensive therapy.

  2016cited by this paper
• WNK signalling pathways in blood pressure regulation

  2016cited by this paper
• Regulation of Renal Electrolyte Transport by WNK and SPAK-OSR1 Kinases.

  2016influential reference
• Potassium and Its Discontents: New Insight, New Treatments.

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  2015cited by this paper
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• SPAK-mediated NCC regulation in response to low-K+ diet.

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• Collecting duct principal cell transport processes and their regulation.

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  2015cited by this paper
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  2015cited by this paper
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  2015cited by this paper
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  2008influential reference
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  2005influential reference
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  2003influential reference
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  2001influential reference

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