Background:The binding partners of the ZASP internal region that is mutated in zaspopathy are not yet known. Results: The internal region of ZASP binds to skeletal muscle ␣-actin, and zaspopathy mutations cause actin disruption. Conclusion: ZASP mutations in the actin-binding domain are deleterious to the muscle Z-disc structure. Significance: ZASP-actin interaction expands the role of ZASP and defines the mechanism of zaspopathy.
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