Mario Nuvolone

This study targets the Sec61 translocon to suppress pathogenic light-chain production in AL amyloidosis, showing that mycolactone and synthetic inhibitors like A317 reduce LC secretion while highlighting the need for client selectivity.

This study uses hiPSC-derived cardiomyocytes to show that cardiotoxic light chains reduce electrophysiological function, while non-toxic light chains have no effect.

This study develops a C. elegans model expressing a human amyloidogenic light chain to study cardiac tropism in AL amyloidosis, finding pharyngeal impairment and oxidative stress similar to human cardiac disease.

This study identifies GLUL upregulation and altered glutamate/glutamine metabolism in prion-infected skeletal muscle, distinct from other neurodegenerative diseases.

This study identifies a conformational fingerprint distinguishing AL from MM light chains using SAXS and MD simulations, revealing a unique H state in AL LCs.

This study investigates the role of cellular prion protein in tau seeding and spreading using Prnp mutant mice and P301S tauopathy models, finding it significant in the latter but not in Alzheimer's sarkosyl-derived samples.

This study develops an ex vivo mouse brain slice model that mimics brain aging and shows prions accelerate aging-related molecular changes.

This paper shows that ASC controls AA amyloidosis by interacting with SAA and that anti-ASC antibodies reduce amyloid loads in mice.

This study investigates the role of PrPC in hippocampal function using PrnpZH3/ZH3 mice, finding behavioral deficits, learning impairment, and increased excitability without enhanced LTP.

The paper shows that glial activation in prion diseases is nonautonomous, requiring neuronal PrPSc to drive neural dysfunction.

The paper identifies early molecular changes in prion-infected mice, suggesting glial pathophysiology as a key driver and potential targets for intervention.

This study finds that Lag3 expression increases in prion infections but does not affect disease progression in knockout mice.