During starvation, a lack of amino acids occurs in cells, which is why greatly enlarged mitochondria are formed. They are much better able to metabolize fats in order to counteract the lack of energy in the cells.

The team gained the knowledge by observing a human cell line that carried a rare hereditary mutation, X-linked myotubular myopathy (XLCNM). In this disorder, which mostly affects boys, a gene on the X chromosome is defective, resulting in a developmental disorder of skeletal muscle.

Original publications:
Jang, W., Puchkov, D., Samso, P., Liang, Y.T., Nadler-Holly, M., Sigrist, S.J., Kintscher, U., Liu, F., Mamchaoui, K., Mouly, V., Haucke, V. (2022) Endosomal lipid signaling reshapes the endoplasmic reticulum to control mitochondrial function. Science, 10.1126/science.abq5209

(2) Samso, P.*, Koch, P.A.*, Posor, Y., Lo, W.T., Belabed, H., Nazare, M., Laporte, J., Haucke, V. (2022) Antagonistic control of active surface integrins by myotubularin and phosphatidylinositol 3-kinase C2b in a myotubular myopathy model. Proc Natl Acad Sci USA 119, e2202236119.

More information:
https://idw-online.de/de/news806728