Protein folding is inherently error prone, especially in the endoplasmic reticulum (ER). Even
with an elaborate network of molecular chaperones and protein folding facilitators …

Lysosomes are the primary catabolic compartments of eukaryotic cells. They degrade
extracellular material that has been internalized by endocytosis and intracellular …

The discovery that heterozygous and homozygous mutations in the gene encoding
progranulin are causally linked to frontotemporal dementia and lysosomal storage disease …

Lysosomes are composed of soluble and transmembrane proteins that are targeted to
lysosomes in a signal-dependent manner. The majority of soluble acid hydrolases are …

The most common inherited form of Frontotemporal Lobar Degeneration (FTLD) known
stems from Progranulin (GRN) mutation and exhibits TDP-43 plus ubiquitin aggregates …

Lysosomal storage disorders, of which more than 40 are known, are caused by the defective
activity of lysosomal proteins, which results in the intra-lysosomal accumulation of …

Sphingolipids and glycosphingolipids are membrane components of eukaryotic cell
surfaces. Their constitutive degradation takes place on the surface of intra-endosomal and …

This review focuses on the pathways that regulate lysosome biogenesis and that are
implicated in numerous degenerative storage diseases, including lysosomal storage …

Lysosomal hydrolases are synthesized in the rough endoplasmic reticulum and specifically
transported through the Golgi apparatus to the trans-Golgi network, from which transport …

Brain-derived neurotrophic factor (BDNF), after activity-dependent secretion from neurons,
modulates critical nervous system functions. Recently, a variant in the human bdnf gene …