Why the study?
While the structure and physiological roles of transverse-tubules are relatively well established, the mechanisms shaping them require further elucidation.
This review highlights the central mechanisms of T-tubule formation and maintenance, and their disruption in inherited muscular disorders like Centronuclear myopathy.
T-tubule biogenesis remains untargetable clinically; leaves open validation of cytosol-zoning regulators in cardiac remodeling.
A transverse-tubule (T-tubule) is an invagination of the plasma membrane penetrating deep into muscle cells. An extensive membrane network of T-tubules is crucial for rapid and synchronized signal transmission from the cell surface to the entire sarcoplasmic reticulum for Ca2+ release, leading to muscle contraction. T-tubules are also indispensable for the formation and positioning of other muscle organelles. Their structure and physiological roles are relatively well established; however, the mechanisms shaping T-tubules require further elucidation. Centronuclear myopathy (CNM), an inherited muscular disorder, accompanies structural defects in T-tubules. Membrane traffic-related genes, including MTM1 (Myotubularin 1), DNM2 (Dynamin 2), and BIN1 (Bridging Integrator-1), were identified as causative genes of CNM. In addition, causative genes for other muscle diseases are also reported to be involved in the formation and maintenance of T-tubules. This review summarizes current knowledge on the mechanisms of how T-tubule formation and maintenance is regulated.
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Kawaguchi et al. (2023) conducted a review in Centronuclear myopathy (CNM) and other muscle diseases. T-tubule formation and maintenance mechanisms was evaluated.
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