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CFTR and TNR-CFTR expression and function in the kidney.

Jackson Souza-Menezes1,2, Geórgia da Silva Feltran3, Marcelo M Morales4

  • 1Laboratório Integrado de Ciências Morfofuncionais, Núcleo em Ecologia e Desenvolvimento Sócio-Ambiental, Centro de Ciências da Saúde, Universidade Federal do Rio de Janeiro, Av. São José do Barreto, 764, Barreto, Macaé, 27965-045, RJ, Brazil. jacksonmenezes@gmail.com.

Biophysical Reviews
|May 17, 2017
PubMed
Summary

The cystic fibrosis transmembrane conductance regulator (CFTR) is found in the kidney and regulates ion transport. A variant, TNR-CFTR, also functions in the kidney, potentially in intracellular organelles.

Keywords:
CFTREndocytosisKidneyPotassium channelSodium channelTNR-CFTR

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Area of Science:

  • Nephrology
  • Molecular Biology
  • Physiology

Background:

  • The cystic fibrosis transmembrane conductance regulator (CFTR) is expressed in the kidney, with mRNA detected across all nephron segments.
  • CFTR protein localizes to the apical surface of proximal and distal tubules, suggesting roles in ion transport and pH regulation within endocytic vesicles.
  • A kidney-specific splicing variant, TNR-CFTR, is also present, particularly in the renal medulla.

Purpose of the Study:

  • To review the proposed functions of CFTR and its splicing variant, TNR-CFTR, in the kidney.
  • To discuss the potential roles of CFTR in ion transport and pH homeostasis.
  • To explore the implications of TNR-CFTR in renal function, especially in the context of cystic fibrosis.

Main Methods:

  • Analysis of existing literature on CFTR and TNR-CFTR expression and function in the kidney.
  • Review of studies investigating CFTR localization in rat and human kidney tissues.
  • Examination of research on the functional capacity of TNR-CFTR in preclinical models and cystic fibrosis patients.

Main Results:

  • CFTR is present in various kidney segments, with higher expression in the cortex and outer medulla.
  • CFTR's localization suggests a role in regulating endosomal pH and ion transport (sodium, chloride, potassium).
  • TNR-CFTR shares functional characteristics with wild-type CFTR and can partially compensate for CFTR deficiency in the renal medulla.

Conclusions:

  • CFTR plays significant roles in kidney physiology, including ion transport and potentially intracellular pH regulation.
  • TNR-CFTR is a functionally relevant variant in the kidney, possibly acting within intracellular organelles.
  • Both CFTR and TNR-CFTR are important targets for understanding kidney function and cystic fibrosis pathology.