Results 81 to 90 of about 243,600 (356)

An Out‐of‐Place Etiology: Recognizing FMR1 Premutation in the Memory Clinic

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT The FMR1 gene premutation (55–200 CGG repeats) is usually associated with a wide range of symptoms and phenotypes within the Fragile X‐tremor/ataxia syndrome (FXTAS), but may also manifest as predominant or isolated cognitive decline. We describe three male patients referred for progressive cognitive impairment and behavioral changes. Standard
Guido Greco, Caterina Motta, Enrica Marchionni, Maria Rosaria D'Apice, Carlangelo Carrese, Giuseppe Novelli, Alessandro Martorana, Chiara Giuseppina Bonomi   +7 more
wiley   +1 more source

Revisiting protein aggregation as pathogenic in sporadic Parkinson and Alzheimer diseases. [PDF]

, 2019
The gold standard for a definitive diagnosis of Parkinson disease (PD) is the pathologic finding of aggregated α-synuclein into Lewy bodies and for Alzheimer disease (AD) aggregated amyloid into plaques and hyperphosphorylated tau into tangles.
Brundin, P, Cambi, F, Espay, AJ, Fasano, A, Fernandez, HH, Gan-Or, Z, Greenamyre, JT, Josephs, KA, Kauffman, MA, Lang, AE, Leverenz, JB, Litvan, I, Marsili, L, Mata, IF, Merola, A, Morgante, F, Perry, G, Sardi, SP, Savica, R, Schwarzschild, MA, Sherer, T, Simon, DK, Standaert, DG, Tanner, CM, Vizcarra, JA, Yamasaki, TR, Zabetian, CP   +26 more
core   +3 more sources

Cutaneous Phosphorylated Alpha‐Synuclein in Lewy Body Dementia

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective To determine the test performance of cutaneous phosphorylated alpha‐synuclein (P‐SYN) in dementia with Lewy bodies (DLB), individuals with reduced Montreal Cognitive Assessment (MoCA) and healthy controls. Methods This is the first subgroup analysis of the Synuclein‐One study, a prospective, blinded study evaluating P‐SYN detection ...
Christopher H. Gibbons, Todd Levine, Charles H. Adler, Bailey Bellaire, Ningshan Wang, Pinky Agarwal, Georgina M. Aldridge, Alexandru Barboi, Daniel Claassen, Virgilio G. H. Evidente, Douglas Galasko, Alejandra Gonzalez‐Duarte, Ramon Gil, Mark Gudesblatt, Stuart H. Isaacson, Horacio Kaufmann, Pravin Khemani, Rajeev Kumar, Guillaume Lamotte, Andy J. Liu, Nikolaus R. McFarland, Mitchell G. Miglis, Adam Reynolds, Gregory A. Sahagian, Marie‐Helene Saint‐Hilaire, Julie B. Schwartzbard, Wolfgang Singer, Michael J. Soileau, Steven Vernino, Patricio Millar Vernetti, Oleg Yerstein, Roy Freeman   +31 more
wiley   +1 more source

Toxoplasma GRA16 attenuates Tau hyperphosphorylation and enhances autophagy in thrombin-treated HT-22 hippocampal neuronal cells

Scientific Reports
This study investigated whether Toxoplasma gondii-derived dense granule protein 16 (GRA16) modulates tau protein to attenuate tau hyperphosphorylation and promotes autophagy to facilitate the removal of tau aggregates.
Seung-Hwan Seo, Do-Won Ham, Ji-Eun Lee, Eun-Hee Shin   +3 more
doaj   +1 more source

Variably Protease‐Sensitive Prionopathy: Two New Cases With Motor Neuron‐Dementia Syndrome

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT We describe two patients with variably protease‐sensitive prionopathy (VPSPr) who developed progressive upper motor neuron symptoms, insomnia, behavioral and cognitive decline, compatible with primary lateral sclerosis associated with frontotemporal dementia (FTD).
María Elena Erro, María Victoria Zelaya, Hasier Eraña, Javier Sánchez Ruiz de Gordoa, Fermín García‐Amigot, Anika Simonovska‐Serra, María Cristina Caballero, Isidre Ferrer, Ellen Gelpi, Ivonne Jericó, Joaquín Castilla   +10 more
wiley   +1 more source

Expression of A152T human tau causes age-dependent neuronal dysfunction and loss in transgenic mice. [PDF]

, 2016
A152T-variant human tau (hTau-A152T) increases risk for tauopathies, including Alzheimer's disease. Comparing mice with regulatable expression of hTau-A152T or wild-type hTau (hTau-WT), we find age-dependent neuronal loss, cognitive impairments, and ...
Craft, Ryan, Davis, Allyson, Djukic, Biljana, Gill, T Michael, Guo, Weikun, Kim, Daniel, Lo, Iris, Maeda, Sumihiro, Masliah, Eliezer, Mucke, Lennart, Ponnusamy, Ravikumar, Taneja, Praveen, Wang, Xin, Yu, Gui-Qiu   +13 more
core   +2 more sources

APP Metabolism Regulates Tau Proteostasis in Human Cerebral Cortex Neurons

Cell Reports, 2015
Accumulation of Aβ peptide fragments of the APP protein and neurofibrillary tangles of the microtubule-associated protein tau are the cellular hallmarks of Alzheimer’s disease (AD).
Steven Moore, Lewis D.B. Evans, Therese Andersson, Erik Portelius, James Smith, Tatyana B. Dias, Nathalie Saurat, Amelia McGlade, Peter Kirwan, Kaj Blennow, John Hardy, Henrik Zetterberg, Frederick J. Livesey   +12 more
doaj   +1 more source

Tau Proteins and Neurofibrillary Degeneration

Brain Pathology, 1991
The paired helical filament is the major fibrous component of neurofibrillary pathology in Alzheimer's disease. Over the last three years evidence has accumulated that the microtubule‐associated protein tau forms an important, if not the sole, constituent of the paired helical filament.
M, Goedert, M G, Spillantini, R A, Crowther   +2 more
openaire   +2 more sources

Neuropsychiatric Symptoms Mimicking Dementia in a Patient Treated With Imatinib

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Tyrosine kinase inhibitors are the cornerstone of chronic myeloid leukemia treatment. Newer agents have more potency and a broader spectrum of action, but also a higher potential for neuropsychiatric side effects. We present a case of a patient on imatinib who developed progressive cognitive, mood, and behavioral alterations.
Ashley Jones, Samuele Bonomi, Keith E. Stockerl‐Goldstein, Randall J. Bateman   +3 more
wiley   +1 more source

Characterization of Clinical Phenotype to Glial Fibrillary Acidic Protein Concentrations in Alexander Disease

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective To determine the concentration of glial fibrillary acidic protein (GFAP) in cerebrospinal fluid (CSF) and plasma in Alexander disease (AxD) and whether GFAP levels are predictive of disease phenotypes. Methods CSF and plasma were collected (longitudinally when available) from AxD participants and non‐AxD controls.
Amy T. Waldman, Asako Takanohashi, Joshua Y. Joung, Geraldine W. Liu, Kaley Arnold, Amy Pizzino, Walter Faig, Sarah Woidill, Sona Narula, Adeline L. Vanderver   +9 more
wiley   +1 more source