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Functional Reconstitution and Channel Activity Measurements of Purified Wildtype and Mutant CFTR Protein
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CFTR structure and function: is there a role in the kidney?

J Souza-Menezes1, M M Morales2,3

  • 1Instituto de Biofísica Carlos Chagas Filho, Universidade Federal do Rio de Janeiro, Macaé, Brazil.

Biophysical Reviews
|May 17, 2017
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Cystic fibrosis (CF) affects the kidney despite normal function, altering urine concentration and protein excretion. The CF transmembrane conductance regulator (CFTR) protein

Keywords:
CFTRChloride channelENaCKidneyNephronROMKTNR-CFTR

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Area of Science:

  • Nephrology
  • Genetics
  • Molecular Biology

Background:

  • Cystic fibrosis (CF) is a genetic disorder caused by CF transmembrane conductance regulator (CFTR) mutations.
  • CFTR is expressed in the kidney, but major renal dysfunction is not typical in CF patients.
  • However, altered urinary protein excretion and urine concentrating/diluting capacity are observed in CF.

Purpose of the Study:

  • To review the structure and function of CFTR.
  • To elucidate the role of CFTR in renal physiology.
  • To explore CFTR modulation by hormones regulating extracellular fluid volume.

Main Methods:

  • Review of existing literature on CFTR in renal physiology.
  • Analysis of studies on CFTR expression and function in kidney tissues.
  • Examination of CFTR's role in ion transport and protein handling in nephrons.

Main Results:

  • CFTR mRNA is present in all nephron segments, with higher abundance in the renal cortex and outer medulla.
  • CFTR protein is detected on the apical surface of proximal and distal tubules in rat kidneys.
  • CFTR transports both Cl- and ATP, potentially influencing Na+ and K+ channels and protein endocytosis.

Conclusions:

  • CFTR plays a significant role in renal physiology beyond chloride transport.
  • Its function in ion transport and potential involvement in protein handling are critical for kidney function.
  • Understanding CFTR's renal role is essential for managing CF patients and exploring therapeutic targets.