Albert Lee

The paper compares proteostasis capacity in two neuronal cell lines by analyzing inclusion formation of an aggregation-prone protein, revealing differences in proteostasis machinery engagement.

This study uses zebrafish models to show that human TDP-43 overexpression in motor neurons causes MND-like phenotypes through gain-of-function mechanisms, with distinct effects based on TDP-43 variant.

This study investigates mitochondrial dysfunction in Machado Joseph disease using multiple models, finding impaired oxidative phosphorylation and altered mitochondrial morphology in mice and zebrafish.

This study shows that sodium butyrate improves symptoms of SCA3 by enhancing autophagy through PKA/AMPK signaling in both cell and zebrafish models.

This study identifies a transient protein folding response in early TDP-43 disease that reduces aggregation, with DNAJB5 as a protective factor, and shows proteomic similarities between mouse and human TDP-43 proteinopathies.

This study shows that SUMO modification affects TDP-43 phase separation and aggregation in zebrafish spinal neurons, linking post-translational changes to ALS pathology.

The paper shows sodium valproate activates the sirtuin pathway, improving movement in a zebrafish model of spinocerebellar ataxia-3.

This study shows riluzole does not improve disease outcomes in a mouse model of ALS with cytoplasmic TDP-43 pathology.

This study examines SFPQ dysregulation in ALS patients, finding increased intron retention, reduced mRNA expression in motor cortex, and cytoplasmic aggregates.

The paper validates and optimizes Bimolecular Fluorescence Complementation (BiFC) in zebrafish to study ALS-linked protein aggregate formation in vivo.

The paper identifies cyclin F interactors using BioID and shows that ALS/FTD-associated cyclin F mutations disrupt paraspeckle protein homeostasis, particularly RBM14 levels in patient tissue.