... their regulation of microtubule dynamics, and the significance for these mechanisms in neuronal development and brain diseases. Microtubules Microtubule-binding proteins Neurodegeneration Neurons Phase separation National Institutes of Health http://dx.doi.org/10.13039/100000002 R21 NS112121...

... explores the prospect of import machinery perturbation as a cause of neurodegeneration instigated by the accumulation of aggregating proteins linked to disease. We found that the aggregation-prone Tau variant (Tau P301L ) reduces the levels of components of the import machinery of the outer (TOM20, encoded...

... contributes to the maintenance of nervous system homeostasis. Defective or dysregulated mitophagy has been implicated in the pathogenesis of neurodegenerative diseases. Autophagy Energy homeostasis Mitochondria Mitophagy Nervous system Neurodegeneration Neurodegenerative diseases Neuron Non...

... manipulation. Autophagy Proteostasis network Neurodegeneration Austrian Science Fund http://dx.doi.org/10.13039/501100002428 P30401-B21 W1261 F79 Horizon 2020 Framework Programme http://dx.doi.org/10.13039/100010661 847548 All living systems must keep...

... of dynein heavy chain 1 confirms that dynein has roles in axonal growth regulation, including axon length sensing, retrograde injury signaling and nerve regeneration. Dynein heavy chain Axonal transport Length sensing Cell size Neurodegeneration Agence Nationale de la Recherche http...

... of the VPS13A–XK complex is the basis of both diseases. Summary: Mutations in VPS13A and XK cause related neurodegenerative disorders. A pathological VPS13A mutation disrupts binding of the VPS13A and XK proteins, suggesting a common basis of both diseases. Neurodegeneration Neuro...

.... Comparison with tomograms of degenerative retina from a mouse model of retinitis pigmentosa illustrates the potential of this method for analyzing disease processes like neurodegeneration at sub-200 nm resolution. As a non-destructive imaging method, PXCT is very suitable for correlative imaging. Within...

... and neurons. Despite its importance in disease, mitophagy is likely dispensable under physiological conditions. This Review explores the in vivo roles of mitophagy in mammalian systems, focusing on the best studied examples – mitophagy in neurodegeneration, cardiomyopathy, metabolism, and red blood cell...

... they contribute to disease development. Ubiquitin HECT E3 ubiquitin ligase Ubiquitylation Protein-protein interactions Protein turnover Cell signaling Cancer Neurological disorders Neurodevelopmental disorders Neurodegeneration National Institutes of Health 10.13039/100000002...

... glycosylphosphatidylinositol (GPI)-anchored proteins and thereby promotes neuronal differentiation both in vitro and in vivo . GDE2 is a prognostic marker in neuroblastoma, while loss of GDE2 leads to progressive neurodegeneration in mice; however, its regulation remains unclear. Here, we report that, in immature neuronal...

... toxicity FUS cellular localisation Neurodegeneration Drosophila Ministry of Education, Culture, Sports, Science and Technology 10.13039/501100001700 17H05699 Ministry of Health, Labour and Welfare 10.13039/501100003478 H24-Soyaku-Sogo-002 Japan Agency for Medical...

... in Parkinson's disease, causes impaired activation of Akt as a result of defects in phospholipid PI(3,4,5)P 3 regulation. Akt Parkinson's disease PINK1 neurodegeneration PIP 3 Irish Research Council for Science, Engineering and Technology 10.13039/501100001596 GOIPG/2015/3467...

... , M. ( 2014 ). PERK mediates eIF2α phosphorylation responsible for BACE1 elevation, CREB dysfunction and neurodegeneration in a mouse model of Alzheimer's disease . Neurobiol. Aging   35 , 2272 - 2281 . 10.1016/j.neurobiolaging.2014.04.031 Dolev , I. , Fogel , H. , Milshtein...

... - 2415 . 10.1038/nprot.2006.356 Lau , A. and Tymianski , M. ( 2010 ). Glutamate receptors, neurotoxicity and neurodegeneration . Pflugers Arch.   460 , 525 - 542 . 10.1007/s00424-010-0809-1 Li , Y. , Yang , X. , Ma , C. , Qiao , J. and Zhang , C...

... , A. and Ciechanover , A. ( 1998 ). The ubiquitin system . Annu. Rev. Biochem.   67 , 425 - 479 . 10.1146/annurev.biochem.67.1.425 Huang , Q. and Figueiredo-Pereira , M. E. ( 2010 ). Ubiquitin/proteasome pathway impairment in neurodegeneration: therapeutic implications . Apoptosis   15...

... strategies for amyloid disorders. Amyloid Autophagy Disaggregase Prion Neurodegeneration National Institutes of Health 10.13039/100000002 T32GM008076 R01GM099836 R21NS090205 R21NS102687 Life Extension Foundation Sanofi 10.13039/100004339 Office...

... cells using a model of neurodegeneration. Overall, this study highlights a role for the Golgi ribbon in pathways central to cellular homeostasis. This article has an associated First Person interview with the first author of the paper. * Author for correspondence ( [email protected]...

... between Mcl-1 levels and disease severity. These results emphasize the potential of Mcl-1 upregulation as an attractive therapeutic strategy for delaying or preventing neurodegeneration in AD. We next investigated whether phosphorylation of Mcl-1 at T92 is responsible for nuclear translocation of Mcl-1...

... integrity of the synapse – mutant NMJs present with progressive structural changes and upregulated autophagy, features commonly observed during ageing and in models of neurodegeneration. Unexpectedly, we find instances of nerve terminal detachment from the muscle fibre; to our knowledge, this is the first...

... as primary targets of α-synuclein, revealing 37 mitochondrial proteins not previously linked to α-synuclein or neurodegeneration pathways. Of these, sideroflexin 3 (SFXN3) was found to be a mitochondrial protein localized to the inner mitochondrial membrane. Loss of SFXN3 did not disturb mitochondrial...