Results 51 to 60 of about 636,708 (294)

Linking neurogenesis, oligodendrogenesis, and myelination defects to neurodevelopmental disruption in primary mitochondrial disorders

FEBS Letters, EarlyView.
Mitochondrial remodeling shapes neural and glial lineage progression by matching metabolic supply with demand. Elevated OXPHOS supports differentiation and myelin formation, while myelin compaction lowers mitochondrial dependence, revealing mitochondria as key drivers of developmental energy adaptation.
Sahitya Ranjan Biswas, Porter L. Tomsick, Alicia M. Pickrell, Paul D. Morton   +3 more
wiley   +1 more source

Impaired Interoceptive Accuracy in Semantic Variant Primary Progressive Aphasia

Frontiers in Neurology, 2017
BackgroundInteroception (the perception of internal bodily sensations) is strongly linked to emotional experience and sensitivity to the emotions of others in healthy subjects.
Charles R. Marshall, Charles R. Marshall, Chris J. D. Hardy, Lucy L. Russell, Camilla N. Clark, Katrina M. Dick, Emilie V. Brotherhood, Rebecca L. Bond, Catherine J. Mummery, Jonathan M. Schott, Jonathan D. Rohrer, James M. Kilner, Jason D. Warren   +12 more
doaj   +1 more source

Human Huntington’s disease pluripotent stem cell-derived microglia develop normally but are abnormally hyper-reactive and release elevated levels of reactive oxygen species

Journal of Neuroinflammation, 2021
Background Neuroinflammation may contribute to the pathogenesis of Huntington’s disease, given evidence of activated microglia and elevated levels of inflammatory molecules in disease gene carriers, even those many years from symptom onset. We have shown
Grace C. O’Regan, Sahar H. Farag, Caroline S. Casey, Alison Wood-Kaczmar, Jennifer M. Pocock, Sarah J. Tabrizi, Ralph Andre   +6 more
doaj   +1 more source

Klotho pathways, myelination disorders, neurodegenerative diseases, and epigenetic drugs [PDF]

, 2020
In this review we outline a rationale for identifying neuroprotectants aimed at inducing endogenous Klotho activity and expression, which is epigenetic action, by definition. Such an approach should promote remyelination and/or stimulate myelin repair by
Chen, Xiaohong, Faller, Douglas V., Glavas, Ioannis P., Harpp, David N., Kanara, Iphigenia, Kodukula, Krishna, Mavrakis, Anastasios N., Moos, Walter H., Pernokas, Julie, Pernokas, Mark, Pinkert, Carl A., Powers, Whitney R., Sampani, Konstantina, Steliou, Kosta, Vavvas, Demetrios G., Zamboni, Robert J.   +15 more
core   +1 more source

The ubiquitin ligase RNF115 is required for the clearance of damaged lysosomes

FEBS Letters, EarlyView.
Upon lysosomal rupture, an E3 ubiquitin ligase RNF115 translocates from the cytosol to the damaged lysosomal membrane. Moreover, RNF115 depletion impairs the clearance of damaged lysosomes, identifying it as a key regulator of lysosomal quality control.
Sae Nakanaga, Toshiki Takahashi, Akiko Kuma, Hiroyuki Kawahara   +3 more
wiley   +1 more source

Retained capacity for perceptual learning of degraded speech in primary progressive aphasia and Alzheimer’s disease

Alzheimer’s Research & Therapy, 2018
Background Processing of degraded speech is a promising model for understanding communication under challenging listening conditions, core auditory deficits and residual capacity for perceptual learning and cerebral plasticity in major dementias. Methods
Chris J. D. Hardy, Charles R. Marshall, Rebecca L. Bond, Lucy L. Russell, Katrina Dick, Cono Ariti, David L. Thomas, Sonya J. Ross, Jennifer L. Agustus, Sebastian J. Crutch, Jonathan D. Rohrer, Doris-Eva Bamiou, Jason D. Warren   +12 more
doaj   +1 more source

Transgenic Zebrafish as a Novel Animal Model to Study Tauopathies and Other Neurodegenerative Disorders in vivo [PDF]

, 2010
Our ageing society is confronted with a dramatic increase in patients suffering from tauopathies such as Alzheimer's disease, frontotemporal dementia and others.
Haass, Christian, Paquet, Dominik, Schmid, Bettina   +2 more
core   +1 more source

AAA+ protein unfoldases—the Moirai of the proteome

FEBS Letters, EarlyView.
AAA+ unfoldases are essential molecular motors that power protein degradation and disaggregation. This review integrates recent cryo‐electron microscopy (cryo‐EM) structures and single‐molecule biophysical data to reconcile competing models of substrate translocation.
Stavros Azinas, Marta Carroni
wiley   +1 more source

Prion degradation pathways: Potential for therapeutic intervention [PDF]

, 2015
Prion diseases are fatal neurodegenerative disorders. Pathology is closely linked to the misfolding of native cellular PrP(C) into the disease-associated form PrP(Sc) that accumulates in the brain as disease progresses. Although treatments have yet to be
Goold, R, McKinnon, C, Tabrizi, SJ
core   +1 more source

pH‐mediated activation of the lysosomal arginine sensor SLC38A9

FEBS Letters, EarlyView.
Cells monitor nutrient levels via the lysosomal transporter SLC38A9 to activate the mechanistic target of rapamycin complex 1 (mTORC1). This study reveals that SLC38A9 function is regulated by pH. We identified histidine 544 as a critical pH sensor that undergoes conformational changes to control amino acid efflux from lysosomes; therefore, it ...
Xuelang Mu, Ampon Sae Her, Tamir Gonen
wiley   +1 more source