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CFTR Activity: A product of CFTR quantity and function

Total CFTR activity can be defined as total ion transport mediated by CFTR protein channels at the cell surface. Total CFTR activity is determined by1:

  • CFTR quantity: the number of CFTR channels at the cell surface
  • CFTR function: the functional ability of each channel to open and transport ions

CFTR quantity and function determine total CFTR activity1-4

Total CFTR activity is determined by total ion transport

Normal CFTR quantity at the cell surface

Normal quantity of CFTR proteins at the cell surface

CFTR quantity is determined by2,3:

  • CFTR synthesis: CFTR gene transcription, proper splicing, and mRNA translation
  • CFTR processing and trafficking: maturation of the CFTR protein and its delivery to the cell surface
  • CFTR surface stability: amount of time a CFTR channel is at the cell surface before being removed and recycled

Normal CFTR function at the cell surface

Normal function of CFTR proteins at the cell surface

CFTR function is determined by2,4:

The importance of proper ion transport to proper hydration

Normal chloride transport through the CFTR protein channel works to maintain salt, fluid, and pH homeostasis in various epithelial tissues.2,5

Learn how normal CFTR activity contributes to proper organ function.

    References:
  1. Sheppard DN, Rich DP, Ostedgaard LS, Gregory RJ, Smith AE, Welsh MJ. Mutations in CFTR associated with mild-disease-form Cl- channels with altered pore properties. Nature. 1993;362(6416):160-164.
  2. Zielenski J. Genotype and phenotype in cystic fibrosis. Respiration. 2000;67(2):117‐133.
  3. Ward CL, Kopito RR. Intracellular turnover of cystic fibrosis transmembrane conductance regulator. J Biol Chem. 1994;269(41):25710-25718.
  4. Wang W, Linsdell P. Conformational change opening the CFTR chloride channel pore coupled to ATP-dependent gating. Biochim Biophys Acta. 2012;1818(3):851-860.
  5. Rowe SM, Miller S, Sorscher EJ. Cystic fibrosis. N Engl J Med. 2005;352(19):1992-2001.