Article
Defective collagen proteostasis and matrix formation in the pathogenesis of lysosomal storage disorders.
Matrix biology : journal of the International Society for Matrix Biology - 1 Oct 2018
Settembre Carmine, Cinque Laura, Bartolomeo Rosa, Di Malta Chiara, De Leonibus Chiara, Forrester Alison
Abstract excerpt
The lysosome is a catabolic organelle devoted to the degradation of cellular components, such as protein complexes and whole or portion of organelles that reach the lysosomes through (macro)autophagy. The lysosomes also function as signaling organelles by controlling the activity of key metabolic kinases, such as the mechanistic target of Rapamycin complex 1 (mTORC1). Lysosome dysfunction has dramatic...
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