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Keywords: Neurodegeneration
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Journal Articles
Dis Model Mech (2022) 15 (6): dmm049447.
Published: 28 June 2022
.... and Amir , S. ( 2017 ). Neurodegeneration and the circadian clock . Front. Aging Neurosci. 9 , 170 . 10.3389/fnagi.2017.00170 Houl , J. H. , Ng , F. , Taylor , P. and Hardin , P. E. ( 2008 ). CLOCK expression identifies developing circadian oscillator neurons...
Includes: Supplementary data
Journal Articles
Dis Model Mech (2021) 14 (11): dmm049092.
Published: 1 December 2021
...) and amyotrophic lateral sclerosis (ALS). This repeat expansion can be translated into dipeptide repeat proteins (DPRs), and distribution of the poly-GR DPR correlates with neurodegeneration in postmortem C9FTD/ALS brains. Here, we assessed poly-GR toxicity in zebrafish embryos, using an annexin A5-based...
Includes: Supplementary data
Journal Articles
Dis Model Mech (2021) 14 (9): dmm047464.
Published: 21 September 2021
... reabsorption Aging Muscle senescence Neurodegeneration Tele-proteostasis National Key Research and Development Program of China 2017YFA0104602 Guangzhou Regenerative Medicine and Health Guangdong Laboratory 2018GZR110105015 2018GZR0201003 National Natural Science...
Journal Articles
Dis Model Mech (2021) 14 (5): dmm048983.
Published: 4 May 2021
.... Summary: This Special Article discusses cutting-edge approaches to counter aberrant phase transitions in disease using protein disaggregase- and RNA-based strategies. Disaggregase Neurodegeneration Phase transition References Aladesuyi Arogundade , O. , Nguyen , S. , Leung...
Journal Articles
Dis Model Mech (2021) 14 (2): dmm047548.
Published: 11 February 2021
... Henstridge , C. M. , Tzioras , M. and Paolicelli , R. C. ( 2019 ). Glial contribution to excitatory and inhibitory synapse loss in neurodegeneration . Front. Cell. Neurosci. 13 , 63 . 10.3389/fncel.2019.00063 Hirano , A. , Donnenfeld , H. , Sasaki , S. and Nakano...
Includes: Supplementary data
Journal Articles
Dis Model Mech (2020) 13 (10): dmm045807.
Published: 30 October 2020
... of replicative stress and accumulated endogenous DNA damage that was accompanied by stabilization of TRP53. Notably, inactivation of Trp53 prevented apoptosis of Atrip -deficient progenitor cells and was sufficient to rescue retinal dysplasia, neurodegeneration and loss of vision. Together, these results reveal...
Includes: Supplementary data
Journal Articles
Dis Model Mech (2020) 13 (1): dmm041426.
Published: 28 January 2020
... generated to specifically report the activity of ATF6, representing a major branch of the endoplasmic reticulum stress pathway with functions in development and disease. ATF6 Endoplasmic reticulum stress Unfolded protein response Zebrafish Neurodegeneration The endoplasmic reticulum (ER...
Includes: Supplementary data
Journal Articles
Dis Model Mech (2020) 13 (2): dmm041947.
Published: 15 November 2019
... and neurodegeneration . Trends Mol. Med.   22 , 687 - 700 . 10.1016/j.molmed.2016.06.008 Kozich , J. J. , Westcott , S. L. , Baxter , N. T. , Highlander , S. K. and Schloss , P. D. ( 2013 ). Development of a dual-index sequencing strategy and curation pipeline for analyzing amplicon...
Includes: Supplementary data
Journal Articles
Dis Model Mech (2019) 12 (6): dmm037184.
Published: 18 June 2019
... the expression of synaptic vesicle proteins in the inner retina via the p -Akt, CaMKII and CREB pathways, increasing F-actin in RGC dendrites. Retinal ganglion cell Neurodegeneration Synapse IOP BDNF Glaucoma is a neurodegenerative disease for which the pathological hallmark is retinal...
Journal Articles
Dis Model Mech (2019) 12 (5): dmm036590.
Published: 23 May 2019
... undermines basic cell function, with potential implications for a variety of genetic sphingolipidoses and adult neurodegenerative conditions. IGF-1 Neurodegeneration Krabbe disease Sphingolipid Akt mTOR Membrane dysfunction Neuroprotection Lipid raft Phosphorylation GSK3β...
Includes: Supplementary data
Journal Articles
Dis Model Mech (2019) 12 (2): dmm037218.
Published: 15 February 2019
...-beta peptide (Aβ) co-expression in the context of distinct human APOE alleles. We found that co-expression of human APOE ε2 with Aβ attenuated Aβ-induced neurodegeneration, whereas expression of the APOE ε4 allele had no effect on neurodegeneration, indicating a loss of neuroprotective capacity...
Journal Articles
Dis Model Mech (2019) 12 (1): dmm035642.
Published: 11 January 2019
... in mice. RPE PEDF MITF Neurodegeneration Retina Retinal degenerations are a group of clinically and genetically heterogeneous disorders characterized by progressive loss of vision due to degeneration of photoreceptors. Retinal degenerative diseases, which include age-related macular...
Includes: Supplementary data
Journal Articles
Dis Model Mech (2018) 11 (9): dmm034165.
Published: 15 August 2018
... LysoTracker staining intensity in neuromasts. Drug screening Liver disease Lysosomal storage disorder Neurodegeneration Niemann-Pick disease type C1 Zebrafish Niemann-Pick disease type C (NPC) is a rare autosomal recessive disease caused by the accumulation of cholesterol and glycolipids...
Includes: Supplementary data
Journal Articles
Dis Model Mech (2018) 11 (6): dmm031286.
Published: 15 June 2018
... association that has yet to be established in humans. We also show that manipulation of environment increases the severity of neurodegeneration in bgm and dbb mutant flies, adding even further to a suite of new candidate ALD disease-causing genes and pathways in humans. Finally, we show that it is a lack...
Includes: Supplementary data
Journal Articles
Dis Model Mech (2018) 11 (3): dmm031161.
Published: 8 March 2018
...Joshua Spurrier; Arvind Kumar Shukla; Kristina McLinden; Kory Johnson; Edward Giniger ABSTRACT Aging is the greatest risk factor for neurodegeneration, but the connection between the two processes remains opaque. This is in part for want of a rigorous way to define physiological age, as opposed...
Includes: Supplementary data
Journal Articles
Dis Model Mech (2017) 10 (12): 1421–1432.
Published: 1 December 2017
... contribute to the pathophysiology of SBA-like leg dysfunction. Our results shed light on the mechanisms underlying the timing and causes of cellular abnormalization, disinhibition of motor neurons and neurodegeneration, thus opening up the possibility of new approaches to prevent neuronal function...
Includes: Supplementary data
Journal Articles
Dis Model Mech (2017) 10 (10): 1253–1260.
Published: 1 October 2017
... and progression, as determined using mouse models and human samples. TTR gene silencing in the preclinical model decreases MMP-14 plasma levels. MMP-14 Familial amyloidotic polyneuropathy Neurodegeneration Neuroinflammation Peripheral nervous system Transthyretin (TTR)-related amyloidosis...
Includes: Supplementary data
Journal Articles
Dis Model Mech (2017) 10 (8): 1027–1038.
Published: 1 August 2017
... , J. , Ratnakaran , N. and Koushika , S. P. ( 2015 ). Neurodegeneration and microtubule dynamics: death by a thousand cuts . Front Cell Neurosci.   9 , 343 . 10.3389/fncel.2015.00343 DuBoff , B. , Götz , J. and Feany , M. B. ( 2012 ). Tau promotes...
Includes: Supplementary data
Journal Articles
Dis Model Mech (2017) 10 (7): 859–868.
Published: 1 July 2017
... of the hippocampal CA1, enhanced apoptosis, and deficits in gait and cognition. Neither line of mice, however, showed extensive TAR DNA-binding protein 43 (TDP-43) pathology or neurodegeneration. Our data suggest that RNA foci pathology is not a good predictor of C9orf72 RAN dipeptide formation, and that RAN...
Includes: Supplementary data
Journal Articles
Dis Model Mech (2017) 10 (6): 691–704.
Published: 1 June 2017
... and pathophysiology of lysosomal storage diseases, and how iPSCs have illuminated the role of lysosomes in more common disorders. Gaucher disease IPSC models Lysosomal enzymes Lysosomal storage disorders Neurodegeneration There are over 50 types of lysosomal storage disease, a class of inherited...