TRPV4 acts as a mitochondrial Ca2+-importer and regulates mitochondrial temperature and metabolism

TRPV4 is associated with the development of neuropathic pain, sensory defects, muscular dystrophies, neuro-degenerative disorders, Charcot Marie Tooth and skeletal dysplasia. In all these cases, mitochondrial abnor-malities are prominent. Here, we demonstrate that TRPV4, localizes to a subpopulation of mitochondria in various cell lines. Improper expression and/or function of TRPV4 induces several mitochondrial abnormalities. TRPV4 is also involved in the regulation of mitochondrial numbers, Ca2+-levels and mitochondrial temperature. Accordingly, several naturally occurring TRPV4 mutations affect mitochondrial morphology and distribution. These findings may help in understanding the significance of mitochondria in TRPV4-mediated channelopathies possibly classifying them as mitochondrial diseases.

Automated summary

TRPV4 is associated with various diseases, and mitochondrial abnormalities play a significant role in these diseases. Improper expression and function of TRPV4 lead to mitochondrial abnormalities, and TRPV4 also regulates mitochondrial numbers, Ca2+ levels, and temperature. Naturally occurring TRPV4 mutations affect mitochondrial morphology and distribution, which helps in understanding TRPV4-mediated channelopathies and classifying them as mitochondrial diseases.