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Keywords: Neurodegeneration
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Journal Articles
In collection:
Mitochondria
J Cell Sci (2023) 136 (13): jcs260993.
Published: 7 July 2023
... 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...
Includes: Supplementary data
Journal Articles
J Cell Sci (2023) 136 (11): jcs260638.
Published: 6 June 2023
... 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...
Journal Articles
J Cell Sci (2023) 136 (10): jcs260888.
Published: 31 May 2023
... are increased compared to those seen in unaffected control brains ( Koopman and Rudiger, 2020 ). Furthermore, studies on AD brains showed higher BAG3 mRNA levels in inhibitory neurons than excitatory ones, the latter being more vulnerable to neurodegeneration, highlighting BAG3 as a potential central node...
Includes: Supplementary data
Journal Articles
In collection:
Motors
J Cell Sci (2023) 136 (5): jcs260220.
Published: 31 October 2022
... as homozygotes, and the adult heterozygous animals have mild to severe neurodegeneration phenotypes ( Terenzio et al., 2018a ), raising the possibility that specific aspects of the phenotype might be due to effects of the point mutation rather than reduction in dynein levels. An allele deletion model allowing...
Includes: Supplementary data
Journal Articles
J Cell Sci (2022) 135 (17): jcs260227.
Published: 8 September 2022
... by The Company of Biologists Ltd 2022 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-acanthocytosis syndromes...
Includes: Supplementary data
Journal Articles
J Cell Sci (2021) 134 (19): jcs258561.
Published: 14 October 2021
.... 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...
Includes: Supplementary data
Journal Articles
J Cell Sci (2021) 134 (19): jcs240465.
Published: 6 October 2021
... 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...
Journal Articles
J Cell Sci (2020) 133 (7): jcs228072.
Published: 7 April 2020
... Ubiquitylation Protein-protein interactions Protein turnover Cell signaling Cancer Neurological disorders Neurodevelopmental disorders Neurodegeneration National Institutes of Health 10.13039/100000002 R15GM126432 Clark University 10.13039/100009217 Xi'an University...
Includes: Supplementary data
Journal Articles
J Cell Sci (2020) 133 (3): jcs235044.
Published: 10 February 2020
... 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...
Includes: Supplementary data
Journal Articles
J Cell Sci (2019) 132 (20): jcs236836.
Published: 23 October 2019
... toxicity FUS cellular localisation Neurodegeneration Drosophila Long non-coding RNAs (lncRNAs) are transcripts longer than 200 nucleotides found throughout the cell that lack protein-coding function ( Lo Piccolo, 2018 ; Sun et al., 2018 ). It has been revealed nuclear-located lncRNAs form...
Includes: Supplementary data
Journal Articles
J Cell Sci (2019) 132 (20): jcs233221.
Published: 22 October 2019
... 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 PINK1 [phosphatase and tensin homologue (PTEN)-induced putative kinase 1] was identified as a gene upregulated...
Includes: Supplementary data
Journal Articles
J Cell Sci (2019) 132 (13): jcs231258.
Published: 1 July 2019
... Flower Neurodegeneration Throughout development and adult life, organisms are subjected to a variety of external insults and endogenous stress, which can affect cell viability and ultimately cause disease. Cell autonomous mechanisms that monitor intrinsic cellular viability, like the DNA repair...
Journal Articles
J Cell Sci (2018) 131 (22): jcs214684.
Published: 19 November 2018
.... and Banker , G. ( 2006 ). Culturing hippocampal neurons . Nat. Protoc.   1 , 2406 - 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...
Includes: Supplementary data
Journal Articles
J Cell Sci (2018) 131 (11): jcs209122.
Published: 12 June 2018
... , 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...
Includes: Supplementary data
Journal Articles
J Cell Sci (2018) 131 (8): jcs189928.
Published: 13 April 2018
... strategies for amyloid disorders. Amyloid Autophagy Disaggregase Prion Neurodegeneration Amyloid fibrils are protein homopolymers that adopt diverse cross-β conformations ( Fig. 1 A). These non-branching fibrils are stabilized via intermolecular contacts between β-strands, which align...
Journal Articles
In collection:
Autophagy
J Cell Sci (2018) 131 (3): jcs211987.
Published: 8 February 2018
... 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 ( pgleeson@unimelb.edu.au...
Includes: Supplementary data
Journal Articles
In collection:
Mitochondria
J Cell Sci (2017) 130 (18): 3023–3039.
Published: 15 September 2017
... 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. * Author for correspondence ( shah23@purdue.edu ) Competing interests The authors declare...
Includes: Supplementary data
Journal Articles
J Cell Sci (2017) 130 (10): 1772–1784.
Published: 15 May 2017
... 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...
Includes: Supplementary data
Journal Articles
In collection:
Mitochondria
J Cell Sci (2017) 130 (2): 325–331.
Published: 15 January 2017
... 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...
Includes: Supplementary data
Journal Articles
J Cell Sci (2017) 130 (1): 71–82.
Published: 1 January 2017
..., are reviewed. Glioma Mechanobiology Mechanotransduction Microenvironment Neurodegeneration Differentiated tissues exhibit distinct mechanical properties ( Fig. 1 ), which direct their structure and function and reflect the composition and architecture of the extracellular matrix (ECM...