...David Mengel; Isabel G. Wellik; Kristen H. Schuster; Sabrina I. Jarrah; Madeleine Wacker; Naila S. Ashraf; Gülin Öz; Matthis Synofzik; Maria do Carmo Costa; Hayley S. McLoughlin ABSTRACT Increased neurofilament light (NfL; NEFL) protein in biofluids is reflective of neurodegeneration and has gained...

... Neurodegeneration Mouse model Ministry of Education, Culture, Sports, Science and Technology http://dx.doi.org/10.13039/501100001700 18H02592 21H02652 RNA-seq was performed according to previous reports ( Bizen et al., 2022 ; Yoshioka et al., 2022 ). RNA was extracted from the wild-type...

... dephosphorylation by Stg/Cdc25 correlates with reduced Tau oligomerization, brain vacuolization and locomotor deficits in flies. Moreover, using a disease mimetic model, we provided evidence that Stg/Cdc25 reduces Tau phosphorylation levels independently of Tau aggregation status and delays neurodegeneration...

.... Circadian Heat shock protein Hsp40 Huntingtin Huntington's disease Neurodegeneration Drosophila LNv Department of Science and Technology, Ministry of Science and Technology, India http://dx.doi.org/10.13039/501100001409 SR/S2/RJN-42/2008 Council for Scientific and Industrial...

...) 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...

... Cabirol-Pol , M.-J. , Khalil , B. , Rival , T. , Faivre-Sarrailh , C. and Besson , M. T. ( 2018 ). Glial lipid droplets and neurodegeneration in a Drosophila model of complex I deficiency . Glia 66 , 874 - 888 . 10.1002/glia.23290 Cagan , R. L. ( 2011...

.... , Giasson , B. I. , Glavis-Bloom , A. , Brewer , M. D. , Shorter , J. , Gitler , A. D. and Yang , X. ( 2014 ). A cellular system that degrades misfolded proteins and protects against neurodegeneration . Mol. Cell 55 , 15 - 30 . 10.1016/j.molcel.2014.04.030 Guo , L...

... 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 frontotemporal lobar degeneration...

... 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...

... author of the paper . ATF6 Endoplasmic reticulum stress Unfolded protein response Zebrafish Neurodegeneration The endoplasmic reticulum (ER) is an important organelle in the cell for biosynthesis, folding and maturation of proteins destined for the cell membrane or the extracellular space...

... , 60 - 67 . 10.1007/s13311-017-0583-2 Klein , H.-U. and De Jager , P. L. ( 2016 ). Uncovering the role of the methylome in dementia and neurodegeneration . Trends Mol. Med.   22 , 687 - 700 . 10.1016/j.molmed.2016.06.008 Kozich , J. J. , Westcott , S...

... chronic IOP elevation in glaucoma. Retinal ganglion cell Neurodegeneration Synapse IOP BDNF 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. © 2019...

... mechanisms underlining GSL-driven neurodegeneration. Krabbe disease (KD) is an LSD caused by deficient activity of galactosyl-ceramidase (GALC) and the toxic accumulation of the GSL psychosine (galactosyl-sphingosine) ( Igisu and Suzuki, 1984 ), and is compounded by irreversible neuronal and glial...

...-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...

... degeneration and blindness ( Ben M'Barek et al., 2017 ; Kolomeyer and Zarbin, 2014 ; Longbottom et al., 2009 ; Mullen and LaVail, 1976 ; Raymond and Jackson, 1995 ; Strauss, 2005 ). RPE PEDF MITF Neurodegeneration Retina Summary: This study reveals that the MITF-PEDF pathway plays...

... for performing in vivo screening of candidate therapeutic compounds by examining LysoTracker staining intensity in neuromasts. Drug screening Liver disease Lysosomal storage disorder Neurodegeneration Niemann-Pick disease type C1 Zebrafish * Author for correspondence ( fdporter...

... 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...

...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...

... 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...

... 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...