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CFTR Function: How normal CFTR proteins work at the cell surface

CFTR proteins function as channels that transport ions through the apical cell membrane of epithelial cells.1

CFTR protein function is determined by channel-open probability and conductance.1,2

Normal CFTR channel-open probability

Status of a single channel over time3*

CFTR channel-open probability

Channel-open probability: the fraction of time that a single CFTR protein channel is open and transporting ions.2

*Based on in vitro experimentation.

Normal CFTR channel conductance

CFTR channel conductance

Channel conductance: rate at which ions move through open channels.2

What determines total CFTR activity? Learn more.

  1. Zielenski J. Genotype and phenotype in cystic fibrosis. Respiration. 2000;67(2):117‐133.
  2. 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.
  3. Bompadre SG, Sohma Y, Li M, Hwang TC. G551D and G1349D, two CF-associated mutations in the signature sequences of CFTR, exhibit distinct gating defects. J Gen Physiol. 2007;129(4):285-298.