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Keywords: Neurodegeneration
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Journal Articles
Dis Model Mech (2021) 14 (5): dmm048983.
Published: 04 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 Protein-misfolding diseases are a pernicious public health problem. Errors...
Journal Articles
Dis Model Mech (2021) 14 (2): dmm047548.
Published: 11 February 2021
... lateral sclerosis and frontotemporal dementia, using the eye as a model and demonstrated axonal cytoskeleton alterations. TDP-43 Amyotrophic lateral sclerosis Frontotemporal lobar degeneration Neurodegeneration Disease model Visual system Amyotrophic lateral sclerosis (ALS) and...
Includes: Supplementary data
Journal Articles
Dis Model Mech (2020) 13 (10): dmm045807.
Published: 30 October 2020
... 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
... zebrafish 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...
Includes: Supplementary data
Journal Articles
Dis Model Mech (2020) 13 (2): dmm041947.
Published: 15 November 2019
Includes: Supplementary data
Journal Articles
Dis Model Mech (2019) 12 (6): dmm037184.
Published: 18 June 2019
... original work is properly attributed. Summary: Application of BDNF increased 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...
Journal Articles
Dis Model Mech (2019) 12 (5): dmm036590.
Published: 23 May 2019
.... U. and Brenman , J. E. ( 2015 ). Lysosomal storage diseases-regulating neurodegeneration . J. Exp. Neurosci.   9 , 81 - 91 . 10.4137/JEN.S25475 Ozes , O. N. , Akca , H. , Mayo , L. D. , Gustin , J. A. , Maehama , T. , Dixon , J. E. and Donner , D. B...
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
... simple eye drops of a PEDF 17-mer peptide can partially prevent Mitf deficiency-associated retinal degeneration in mice. RPE PEDF MITF Neurodegeneration Retina Retinal degenerations are a group of clinically and genetically heterogeneous disorders characterized by progressive loss of...
Includes: Supplementary data
Journal Articles
Dis Model Mech (2018) 11 (9): dmm034165.
Published: 15 August 2018
... accumulation in late endosomes/lysosomes causes neurodegeneration and is prevented by driving cholesterol export from this compartment . J. Neurosci.   31 , 9404 - 9413 . 10.1523/JNEUROSCI.1317-11.2011 Boer , E. F. , Jette , C. A. and Stewart , R. A. ( 2016 ). Neural crest migration and...
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 of...
Includes: Supplementary data
Journal Articles
Dis Model Mech (2018) 11 (3): dmm031161.
Published: 08 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 to...
Includes: Supplementary data
Journal Articles
Dis Model Mech (2017) 10 (12): 1421–1432.
Published: 01 December 2017
....2015.10.051 Neural tube defect Chicken model Synaptic inputs GABAergic transmission Neurodegeneration Motor dysfunction Spina bifida aperta (SBA), also known as myelomeningocele, is one of the most common congenital malformations, causing lifelong disability. SBA develops in ∼1/1000...
Includes: Supplementary data
Journal Articles
Dis Model Mech (2017) 10 (10): 1253–1260.
Published: 01 October 2017
...-14 plasma levels. MMP-14 Familial amyloidotic polyneuropathy Neurodegeneration Neuroinflammation Peripheral nervous system Transthyretin (TTR)-related amyloidosis is the most common form of hereditary autosomic dominant systemic amyloidoses; TTR point mutations lead to the...
Includes: Supplementary data
Journal Articles
Dis Model Mech (2017) 10 (8): 1027–1038.
Published: 01 August 2017
... Drosophila . Proc. Natl. Acad. Sci. USA   105 , 14503 - 14508 . 10.1073/pnas.0803998105 Dubey , 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...
Includes: Supplementary data
Journal Articles
Dis Model Mech (2017) 10 (7): 859–868.
Published: 01 July 2017
... 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: 01 June 2017
... ( Huang et al., 2011 ; Lemonnier et al., 2011 ; Tiscornia et al., 2013 ). Gaucher disease IPSC models Lysosomal enzymes Lysosomal storage disorders Neurodegeneration Summary: This Review discusses how induced pluripotent stem cells (iPSCs) provide new opportunities to explore the...
Journal Articles
Dis Model Mech (2017) 10 (5): 581–595.
Published: 01 May 2017
... attributed. Summary: The receptor tyrosine kinase Tyro3 improves weight and survival in the anorexia mouse model, and is one of the few factors known to sustain appetite regulatory circuitry. Eating disorder RNA localization Appetite regulation Neurodegeneration Npy Modifier Transgenic...
Includes: Supplementary data
Journal Articles
Dis Model Mech (2017) 10 (5): 537–549.
Published: 01 May 2017
...., 2013 ). A neuroinflammatory response is common to all forms of ALS and most likely modulates the process of neurodegeneration ( Philips and Robberecht, 2011 ). Cell type-specific transgenic mouse models, using the Cre-Lox system to excise the mutant SOD1 transgene from specific cell-types, have...
Journal Articles
Dis Model Mech (2017) 10 (1): 53–62.
Published: 01 January 2017
... embryonic lethal, whereas heterozygous Slc33a1 mutant mice ( Slc33a1 wt/mut ) exhibited behavioral abnormalities and central neurodegeneration, which is consistent with hereditary spastic paraplegia (HSP) phenotypes. Importantly, we found an upregulation of BMP signaling in the nervous system and mouse...
Includes: Supplementary data