The retromer complex is a vital element of the endosomal protein sorting machinery that is
conserved across all eukaryotes. Retromer is most closely associated with the endosome-to …

Retromer is a protein assembly that has a central role in endosomal trafficking, and retromer
dysfunction has been linked to a growing number of neurological disorders. First linked to …

Lysosomes critically rely on bis (monoacylglycero) phosphate (BMP) to stimulate lipid
catabolism, cholesterol homeostasis, and lysosomal function. Alterations in BMP levels in …

In childhood the neuronal ceroid lipofuscinoses (NCL) are the most frequent lysosomal
diseases and the most frequent neurodegenerative diseases but, in adulthood, they …

Lysosomal failure underlies pathogenesis of numerous congenital neurodegenerative
disorders and is an early and progressive feature of Alzheimer's disease (AD) pathogenesis …

The fatal, primarily childhood neurodegenerative disorders, neuronal ceroid lipofuscinoses
(NCLs), are currently associated with mutations in 13 genes. The protein products of these …

Sortilin is a post-Golgi trafficking receptor homologous to the yeast vacuolar protein sorting
receptor 10 (VPS10). The VPS10 motif on sortilin is a 10-bladed β-propeller structure …

In familial neurodegenerative disorders, protein aggregates form continuously because of
genetic mutations that drive the synthesis of truncated or unfolded proteins. The oxidative …

Lysosomes are central organelles for cellular degradation and energy metabolism.
Neuronal ceroid lipofuscinoses (NCLs) are a group of the most common neurodegenerative …

Neuronal ceroid lipofuscinosis (NCL) comprises∼ 13 genetically distinct lysosomal
disorders primarily affecting the central nervous system. Here we report successful …