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- W2912195060 abstract "Abstract Transmembrane collagen XIII has been linked to maturation of the musculoskeletal system. Its absence in mice ( Col13a1 −/− ) results in impaired neuromuscular junction ( NMJ ) differentiation and function, while transgenic overexpression ( Col13a1 oe ) leads to abnormally high bone mass. Similarly, loss‐of‐function mutations in COL 13A1 in humans produce muscle weakness, decreased motor synapse function and mild dysmorphic skeletal features. Here, analysis of the exogenous overexpression of collagen XIII in various muscles revealed highly increased transcript and protein levels, especially in the diaphragm. Unexpectedly, the main location of exogenous collagen XIII in the muscle was extrasynaptic, in fibroblast‐like cells, while some motor synapses were devoid of collagen XIII , possibly due to a dominant negative effect. Concomitantly, phenotypical changes in the NMJ s of the Col13a1 oe mice partly resembled those previously observed in Col13a1 −/− mice. Namely, the overall increase in collagen XIII expression in the muscle produced both pre‐ and postsynaptic abnormalities at the NMJ , especially in the diaphragm. We discovered delayed and compromised acetylcholine receptor ( AC hR) clustering, axonal neurofilament aggregation, patchy acetylcholine vesicle ( AC hV) accumulation, disrupted adhesion of the nerve and muscle, Schwann cell invagination and altered evoked synaptic function. Furthermore, the patterns of the nerve trunks and AC hR clusters in the diaphragm were broader in the adult muscles, and already prenatally in the Col13a1 oe mice, suggesting collagen XIII involvement in the development of the neuromuscular system. Overall, these results confirm the role of collagen XIII at the neuromuscular synapses and highlight the importance of its correct expression and localization for motor synapse formation and function." @default.
- W2912195060 created "2019-02-21" @default.
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- W2912195060 date "2019-02-08" @default.
- W2912195060 modified "2023-09-25" @default.
- W2912195060 title "Correct expression and localization of collagen XIII are crucial for the normal formation and function of the neuromuscular system" @default.
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- W2912195060 doi "https://doi.org/10.1111/ejn.14346" @default.
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