David Vı́lchez

Anle138b ameliorates pathological phenotypes in mouse and cellular models of Huntington's disease

Anle138b reduces mHTT aggregates and improves disease phenotypes in HD cell and mouse models.

Prion-like domains control plastid targeting of PEPSI effectors and PEPSI6-mediated modulation of DXR during root symbiosis

This paper identifies prion-like domains (PrLDs) as noncanonical plastid-targeting elements in PEPSI effectors and shows PEPSI6 modulates DXR during root symbiosis.

The tumor suppressor CYLD acts as a deubiquitinase for mTOR to constrain its activity

This study identifies CYLD as a deubiquitinase that inhibits mTOR activity by removing K63-linked ubiquitin chains, linking its loss to mTOR hyperactivation and disease.

C9orf72 ALS-causing mutations lead to nucleoporin Nup107 aggregation and subsequent pathological changes

This study shows C9orf72 ALS mutations cause Nup107 aggregation in motor neurons, leading to pathological changes and reduced lifespan in worm models.

The anti-leprosy drug clofazimine reduces polyQ toxicity through activation of PPARγ

The paper identifies clofazimine as a drug that reduces polyQ toxicity by activating PPARγ, improving mitochondrial function in models of polyQ diseases.

SPTF-3/SP1 orchestrates mitochondrial biogenesis upon ribosomal stress and acute starvation

The paper identifies SPTF-3 as a stress-induced transcription factor regulating mitochondrial biogenesis and UPRmt in C. elegans, with its mammalian homolog SP1 showing conserved function in nutrient deprivation.

Neuroprotective effects of hepatoma-derived growth factor in models of Huntington's disease

This study shows that hepatoma-derived growth factor (HDGF) has neuroprotective effects in Huntington's disease models by reducing mutant Huntingtin toxicity and aggregation.