ETI corrector therapy suppresses mitochondrial respiration independently of CFTR mutational status.

doi:10.1016/j.bbrc.2026.153848

Article

ETI corrector therapy suppresses mitochondrial respiration independently of CFTR mutational status.

Biochemical and biophysical research communications - 2 Jul 2026

Jha Anupma, Mishra Sanjay K, Zhang Yuxun, Bharathi Sivakama, Goetzman Eric S

Abstract excerpt

The relationship between CFTR mutational status and cellular energy metabolism remains unresolved. Using continuous long-term respirometry via the Resipher platform, we demonstrate that the Class II CFTR misfolding mutations ΔF508, G85E, and P67L all significantly increase cellular respiration in isogenic Fisher rat thyroid (FRT) and human bronchial epithelial (HBE) cells, consistent with the constitutive energy...

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ETI corrector therapy suppresses mitochondrial respiration independently of CFTR mutational status.

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