N. Raimundo

Loss of the lysosomal protein CLN3 modifies the lipid content of the nuclear envelope leading to DNA damage and activation of YAP1 pro-apoptotic signaling

This study shows that loss of CLN3 disrupts lysosomal lipid metabolism, leading to nuclear envelope lipid changes, DNA damage, and YAP1-driven apoptosis in Batten disease models.

Up-regulation of cholesterol synthesis by lysosomal defects requires a functional mitochondrial respiratory chain

The paper identifies that lysosomal defects up-regulate cholesterol synthesis via mitochondrial respiratory chain interactions and Ca2+ retention, while mitochondrial defects down-regulate it.

Functional peroxisomes are required for heat shock-induced hormesis in Caenorhabditis elegans

This study shows that functional peroxisomes are necessary for heat shock-induced hormesis and the canonical heat shock response in C. elegans.

Amyloid-like aggregates cause lysosomal defects in neurons via gain-of-function toxicity

This study shows that amyloid-like aggregates impair lysosomal function in neurons by sequestering AP-31, leading to neurotoxicity.

Impaired lysosomal acidification triggers iron deficiency, necrotic cell death and inflammation in vivo

This study shows that impaired lysosomal acidification causes iron deficiency, mitochondrial dysfunction, necrotic cell death, and inflammation in mice.

Mitochondrial biogenesis is transcriptionally repressed in lysosomal lipid storage diseases

This study identifies a transcriptional repression of mitochondrial biogenesis in lysosomal storage diseases through KLF2 and ETV1, showing their roles in disease mechanisms.