Journal
AUTOPHAGY
Volume 5, Issue 2, Pages 228-229Publisher
TAYLOR & FRANCIS INC
DOI: 10.4161/auto.5.2.7390
Keywords
chondrocytes; macroautophagy; lysosomes; LSD; skeletal abnormalities
Categories
Funding
- NIAMS NIH HHS [R01 AR043655-05] Funding Source: Medline
- Telethon [TGM06C01] Funding Source: Medline
Ask authors/readers for more resources
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 (Sump), 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.
Authors
I am an author on this paper
Click your name to claim this paper and add it to your profile.
Reviews
Recommended
No Data Available