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- W2079480541 abstract "Macroautophagy (a.k.a. autophagy) is a cellular process aimed at the recycling of proteins and organelles that is achieved when autophagosomes fuse with lysosomes. Accordingly, lysosomal dysfunctions are often associated with impaired autophagy. We demonstrated that inactivation of the sulfatase modifying factor 1 gene (Sumf1), a gene mutated in multiple sulfatase deficiency (MSD), causes glycosaminoglycans (GAGs) to accumulate in lysosomes, which in turn disrupts autophagy. We utilized a murine model of MSD to study how impairment of this process affects chondrocyte viability and thus skeletal development." @default.
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- W2079480541 date "2009-02-16" @default.
- W2079480541 modified "2023-09-26" @default.
- W2079480541 title "Self-eating in skeletal development: Implications for lysosomal storage disorders" @default.
- W2079480541 doi "https://doi.org/10.4161/auto.5.2.7390" @default.
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