Title: T-tubule biogenesis and triad formation in skeletal muscle and implication in human diseases | Skeletal Muscle | Full Text
Headings: T-tubule biogenesis and triad formation in skeletal muscle and implication in human diseases
T-tubule biogenesis and triad formation in skeletal muscle and implication in human diseases
Abstract
Introduction
T-tubule plasticity
Morphological aspects of triad biogenesis
Sarcoplasmic reticulum
Transverse tubule
Molecular mechanisms involved in T-tubule biogenesis and triad formation and maintenance
Table 1 Proteins implicated in triad organization, roles and associated human diseasesa
Caveolin 3
Amphiphysin 2 (BIN1)
Dysferlin
Mitsugumins
Junctophilins
Myotubularin
The RyR-DHPR complex
Conclusion
References
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Content: The close association of one T-tubule with two terminal cisternae on both sides of the tubule forms the triad (Figure 1 ). Figure 1
Triad organization in skeletal muscle. Left: Electron micrograph of a triad junction. A central T-tubule is flanked on both sides by a terminal cisternae element from the sarcoplasmic reticulum. Arrows indicate electron-dense junctional feet corresponding to the ryanodine receptor-dihydhropyridine receptor complex. Right: Schematic representation of a mammalian muscle sarcomere and surrounding membranes. T-tubules shown in gray are specialized invaginations of the sarcolemma. The elaborated sarcoplasmic reticulum network is shown in blue.