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Schematic representation of the dysregulatory events in the homeostasis of U snRNPs. Dysregulation of Sm proteins during U snRNP assembly causes cellular proteotoxicity. Early phase plCln deficiency leads to degradation of Sm proteins via autophagy and mislocalisation of unassembled and/or misassembled Sm proteins. Late phase SMN deficiency leads to accumulation of Sm proteins over plCln (Figure taken from [53]).
