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Updated: Feb 17, 2026
In vivo Evaluation of Mucociliary Clearance in Mice
Published on: December 18, 2020
Loss of CDKL5 disrupts respiratory function in mice
1Department of Biological Sciences, National Sun Yat-sen University, Kaohsiung 804, Taiwan; Center for Neuroscience, National Sun Yat-sen University, Kaohsiung 804, Taiwan; Institute of Medical Science and Technology, National Sun Yat-sen University, Kaohsiung 804, Taiwan; Department of Biomedical Science and Environmental Biology, Kaohsiung Medical University, Kaohsiung 804, Taiwan; Doctoral Degree Program in Marine Biotechnology, National Sun Yat-sen University and Academia Sinica, Taiwan.
Abstract:
Cyclin-dependent kinase-like 5 (CDKL5) is an X-linked gene encoding a serine-threonine kinase that is highly expressed in the central nervous system. Mutations in CDKL5 cause neurological and psychiatric symptoms, including early-onset seizures, motor dysfunction, autistic features and sleep breathing abnormalities in patients. It remains to be addressed whether loss of CDKL5 causes respiratory dysfunction in mice. Here, we examined the respiratory pattern of male Cdkl5-/y mice at 1-3 months of age during resting breathing and respiratory challenge (i.e., hypoxia and hypercapnia) via whole body plethysmography. The results demonstrated that the resting respiratory frequency and tidal volume of Cdkl5-/y mice was unaltered compared to that of WT mice at 1 month of age. However, these mutant mice exhibit transient reduction in tidal volume during respiratory challenge even the reduction was restored at 2 months of age. Notably, the sigh-breathing pattern was changed in Cdkl5-/y mice, showing a transient reduction in sigh volume at 1-2 month of age and long-term attenuation of peak expiratory airflow from 1 to 3 month of age. Therefore, loss of CDKL5 causes breathing deficiency, supporting a CDKL5-mediated regulation of respiratory function in mice.
