Lysosome: The Story Beyond the Storage

Since Christian de Duve first described the lysosome in the 1950s, it has been generally presented as a membrane-bound compartment containing acid hydrolases that enables the cell to degrade molecules without being digested by autolysis. For those working on the field of lysosomal storage disorders,...

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Veröffentlicht in:Journal of Inborn Errors of Metabolism and Screening 2016, Vol.4, p.232640981667943
Hauptverfasser: Matte, Ursula, Pasqualim, Gabriela
Format: Artikel
Sprache:eng
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Zusammenfassung:Since Christian de Duve first described the lysosome in the 1950s, it has been generally presented as a membrane-bound compartment containing acid hydrolases that enables the cell to degrade molecules without being digested by autolysis. For those working on the field of lysosomal storage disorders, the lack of one such hydrolase would lead to undegraded or partially degraded substrate storage inside engorged organelles disturbing cellular function by yet poorly explored mechanisms. However, in recent years, a much more complex scenario of lysosomal function has emerged, beyond and above the cellular “digestive” system. Knowledge on how the impairment of this organelle affects cell functioning may shed light on signs and symptoms of lysosomal disorders and open new roads for therapy.
ISSN:2326-4098
2326-4594
2326-4594
DOI:10.1177/2326409816679431