Transcription factors KLF2 and ETV1 repress the transcriptional program of mitochondrial biogenesis, resulting in impaired mitochondrial function in lysosomal storage diseases.

Lysosomes are highly enriched in chloride, which is essential for their degradative function.

Sphingosine acts as a lipid messenger molecule in intracellular signaling networks that are relevant to lysosomal function and Niemann-Pick disease.

The malaria parasite Niemann-Pick Type C1-related protein is an important new antimalarial target.

Sterol kinetics and cell-based assays reveal a heretofore unknown step in cholesterol trafficking through the endolysosomal compartment, involving a direct functional interaction between NPC2 and lysosbisphosphatidic acid.

Sterol transport by Niemann-Pick type C proteins induces the expansion of raft-like domains in the yeast vacuole, enabling engulfment of lipid droplets by microautophagy.

LAMP proteins, the major glycoproteins of the lysosome membrane, bind cholesterol directly and specifically, and interact with NPC1 and NPC2 proteins as part of the lysosomal cholesterol export process.

Molecular analysis of cholesterol transport by NPC1 and NPC1L1 proteins reveals that cholesterol likely moves through these transporters, and ezetimibe blocks NPC1L1 by binding at the interface between multiple domains.

NPC1 is a genetic determinant of filovirus susceptibility in bats, and some variations in bat NPC1 may reflect host adaptations to reduce filovirus replication and virulence.