Lysosome: The Story Beyond the Storage
J. inborn errors metab. screen
;
4: e160044, 2016. graf
Artículo
en Inglés
|
LILACS-Express
| LILACS
| ID: biblio-1090907
ABSTRACT
Abstract 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.
Texto completo:
Disponible
Índice:
LILACS (Américas)
Idioma:
Inglés
Revista:
J. inborn errors metab. screen
Asunto de la revista:
Medicina Cl¡nica
/
Patologia
Año:
2016
Tipo del documento:
Artículo
País de afiliación:
Brasil
Institución/País de afiliación:
Hospital de Clínicas de Porto Alegre/BR
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