King Faisal Yambire, Lorena Fernandez-Mosquera ... Nuno Raimundo
Transcription factors KLF2 and ETV1 repress the transcriptional program of mitochondrial biogenesis, resulting in impaired mitochondrial function in lysosomal storage diseases.
Mariana I Tsap, Andriy S Yatsenko ... Halyna R Shcherbata
Drosophila model reveals that NTE/SWS-associated neurodegeneration is a lysosomal storage disorder accompanied by a leaky brain permeability barrier, abnormal fatty acid metabolism, and inflammation.
Xiang Wang, Vitaliy V Bondar ... Anastasia G Henry
Rab12 was identified as a key modulator of LRRK2-dependent Rab phosphorylation that mediates the recruitment of LRRK2 to lysosomes in response to damage.
The AAA-ATPase VCP sustains sarcoplasmic proteostasis, in part, by controlling autophagosome-lysosome fusion and the integrity of a dynamic tubular lysosomal network.
Leslie A McCauliff, Annette Langan ... Judith Storch
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.
David Massa López, Melanie Thelen ... Markus Damme
The so far uncharacterized lysosomal transporter protein MFSD1 is essential for liver homeostasis and needs the highly glycosylated GLMP protein as an accessory subunit for stability.
King Faisal Yambire, Christine Rostosky ... Nuno Raimundo
Impaired lysosomal acidification results in retention of iron inside lysosomes, triggering functional iron deficiency, dysfunctional mitochondria (especially mtDNA loss), and inflammation in vivo in a mouse model of lysosomal disease.