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Schematic representation of the events that lead to organ pathogenesis downstream of NEU1 deficiency. In absence of NEU1, unprocessed LAMP1 accumulation leads to increased number of lysosomes at the PM resulting in exacerbated lysosomal exocytosis. The abnormal release of lysosomal content extracellularly causes ECM and PM remodeling, changes cell characteristics and ultimately causes organ pathogenesis.
