Zfp106 functions as an RNA binding protein, binds directly to GGGGCC RNA repeats, is required in motor neurons to prevent ALS-like neurodegeneration in mice, and can suppress neurotoxicity in an established fly model of ALS.
Peter M LoCoco, April L Risinger ... William P Clarke
Stimulation of the rate-limiting enzyme in the NAD salvage pathway protected against neurotoxicity and subsequent peripheral neuropathy associated with the widely utilized anticancer drug, paclitaxel.
Axon guidance genes are conserved regulators of neurodegeneration in Drosophila melanogaster and human inducible motor neuron models of UBQLN2-associated amyotrophic lateral sclerosis.
Ahmed M Malik, Roberto A Miguez ... Sami J Barmada
The deposition of Matrin 3, a DNA/RNA binding protein implicated in ALS and FTD, results in neurodegeneration dependent upon its localization, self-association, and ability to bind nucleic acids.
Oleksandr Yagensky, Mahdokht Kohansal-Nodehi ... John JE Chua
Increased levels of brain Hebp1 starting from the presymptomatic stage of Alzheimer’s disease contributes to progressive neuronal loss by triggering mitochondrial-dependent apoptosis in neurons exposed to elevated heme.
A novel auto-inhibitory mechanism regulates the functional activity of the cellular prion protein, PrPC, providing for the first time a coherent molecular model for both its pathological and physiological effects.