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Acta Neuropathologica Communications

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https://www.readbyqxmd.com/read/27908295/dopamine-2-receptor-extracellular-n-terminus-regulates-receptor-surface-availability-and-is-the-target-of-human-pathogenic-antibodies-from-children-with-movement-and-psychiatric-disorders
#1
Nese Sinmaz, Fiona Tea, Deepti Pilli, Alicia Zou, Mazen Amatoury, Tina Nguyen, Vera Merheb, Sudarshini Ramanathan, Sandra T Cooper, Russell C Dale, Fabienne Brilot
Anti-Dopamine-2 receptor (D2R) antibodies have been recently identified in a subgroup of children with autoimmune movement and psychiatric disorders, however the epitope(s) and mechanism of pathogenicity remain unknown. Here we report a major biological role for D2R extracellular N-terminus as a regulator of receptor surface availability, and as a major epitope targeted and impaired in brain autoimmunity. In transfected human cells, purified anti-D2R antibody from patients specifically and significantly reduced human D2R surface levels...
December 1, 2016: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/27894339/the-identification-of-human-pituitary-adenoma-initiating-cells
#2
Branavan Manoranjan, Sujeivan Mahendram, Saleh A Almenawer, Chitra Venugopal, Nicole McFarlane, Robin Hallett, Thusyanth Vijayakumar, Almunder Algird, Naresh K Murty, Doron D Sommer, John P Provias, Kesava Reddy, Sheila K Singh
Classified as benign central nervous system (CNS) tumors, pituitary adenomas account for 10% of diagnosed intracranial neoplasms. Although surgery is often curative, patients with invasive macroadenomas continue to experience significant morbidity and are prone to tumor recurrence. Given the identification of human brain tumor-initiating cells (TICs) that initiate and maintain tumor growth while promoting disease progression and relapse in multiple CNS tumors, we investigated whether TICs also drive the growth of human pituitary adenomas...
November 28, 2016: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/27884214/neuronal-and-glial-changes-in-the-brain-resulting-from-explosive-blast-in-an-experimental-model
#3
James A Goodrich, Jung H Kim, Robert Situ, Wesley Taylor, Ted Westmoreland, Fu Du, Steven Parks, Geoffrey Ling, Jung Y Hwang, Amedeo Rapuano, Faris A Bandak, Nihal C de Lanerolle
Mild traumatic brain injury (mTBI) is the signature injury in warfighters exposed to explosive blasts. The pathology underlying mTBI is poorly understood, as this condition is rarely fatal and thus postmortem brains are difficult to obtain for neuropathological studies. Here we report on studies of an experimental model with a gyrencephalic brain that is exposed to single and multiple explosive blast pressure waves. To determine injuries to the brain resulting from the primary blast, experimental conditions were controlled to eliminate any secondary or tertiary injury from blasts...
November 24, 2016: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/27884177/lrrk2-contributes-to-monocyte-dysregulation-in-parkinson-s-disease
#4
LETTER
Corinna Bliederhaeuser, Lisa Zondler, Veselin Grozdanov, Wolfgang P Ruf, David Brenner, Heather L Melrose, Peter Bauer, Albert C Ludolph, Frank Gillardon, Jan Kassubek, Jochen H Weishaupt, Karin M Danzer
No abstract text is available yet for this article.
November 24, 2016: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/27863507/transgenic-mice-overexpressing-the-als-linked-protein-matrin-3-develop-a-profound-muscle-phenotype
#5
Christina Moloney, Sruti Rayaprolu, John Howard, Susan Fromholt, Hilda Brown, Matt Collins, Mariela Cabrera, Colin Duffy, Zoe Siemienski, Dave Miller, Maurice S Swanson, Lucia Notterpek, David R Borchelt, Jada Lewis
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder of upper and lower motor neurons. Mutations in the gene encoding the nuclear matrix protein Matrin 3 have been found in familial cases of ALS, as well as autosomal dominant distal myopathy with vocal cord and pharyngeal weakness. We previously found that spinal cord and muscle, organs involved in either ALS or distal myopathy, have relatively lower levels of Matrin 3 compared to the brain and other peripheral organs in the murine system...
November 18, 2016: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/27855725/review-of-ryr1-pathway-and-associated-pathomechanisms
#6
REVIEW
Jessica W Witherspoon, Katherine G Meilleur
Ryanodine receptor isoform-1 (RyR1) is a major calcium channel in skeletal muscle important for excitation-contraction coupling. Mutations in the RYR1 gene yield RyR1 protein dysfunction that manifests clinically as RYR1-related congenital myopathies (RYR1-RM) and/or malignant hyperthermia susceptibility (MHS). Individuals with RYR1-RM and/or MHS exhibit varying symptoms and severity. The symptoms impair quality of life and put patients at risk for early mortality, yet the cause of varying severity is not well understood...
November 17, 2016: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/27842611/tau-pathology-in-aged-cynomolgus-monkeys-is-progressive-supranuclear-palsy-corticobasal-degeneration-but-not-alzheimer-disease-like-ultrastructural-mapping-of-tau-by-edx
#7
Toshiki Uchihara, Kentaro Endo, Hiromi Kondo, Sachi Okabayashi, Nobuhiro Shimozawa, Yasuhiro Yasutomi, Eijiro Adachi, Nobuyuki Kimura
Concomitant deposition of amyloid -beta protein (Aβ) and neuronal tau as neurofibrillary tangles in the human brain is a hallmark of Alzheimer disease (AD). Because these deposits increase during normal aging, it has been proposed that aging brains may also undergo AD-like changes. To investigate the neuropathological changes that occur in the aging primate brain, we examined 21 brains of cynomolgus monkeys (7-36 years old) for Aβ- and tau-positive lesions. We found, 1) extensive deposition of Aβ in brains of cynomolgus monkeys over 25 years of age, 2) selective deposition of 4-repeat tau as pretangles in neurons, and as coiled body-like structures in oligodendroglia-like cells and astrocytes, 3) preferential distribution of tau in the basal ganglia and neocortex rather than the hippocampus, and 4) age-associated increases in 30-34 kDa AT8- and RD4-positive tau fragments in sarkosyl-insoluble fractions...
November 14, 2016: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/27842602/rubidium-and-potassium-levels-are-altered-in-alzheimer-s-disease-brain-and-blood-but-not-in-cerebrospinal-fluid
#8
Blaine R Roberts, James D Doecke, Alan Rembach, L Fernanda Yévenes, Christopher J Fowler, Catriona A McLean, Monica Lind, Irene Volitakis, Colin L Masters, Ashley I Bush, Dominic J Hare
Loss of intracellular compartmentalization of potassium is a biochemical feature of Alzheimer's disease indicating a loss of membrane integrity and mitochondrial dysfunction. We examined potassium and rubidium (a biological proxy for potassium) in brain tissue, blood fractions and cerebrospinal fluid from Alzheimer's disease and healthy control subjects to investigate the diagnostic potential of these two metal ions. We found that both potassium and rubidium levels were significantly decreased across all intracellular compartments in the Alzheimer's disease brain...
November 14, 2016: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/27842578/an-autopsy-confirmed-case-of-progressive-supranuclear-palsy-with-predominant-postural-instability
#9
Carolin Kurz, Georg Ebersbach, Gesine Respondek, Armin Giese, Thomas Arzberger, Günter Ulrich Höglinger
Postural instability and supranuclear gaze palsy represent the key symptoms of Richardson's syndrome, the most frequent clinical manifestation of progressive supranuclear palsy (PSP). However, a proportion of PSP patients never develops ocular motor symptoms, which prevents clinicians from establishing the diagnosis during lifetime according to current diagnostic criteria. We present one instructive autopsy-confirmed PSP case with prospective video-documented clinical course, showing striking temporal divergence of initially present postural instability and delayed development of ocular motor dysfunction...
November 14, 2016: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/27809932/tunneling-nanotube-tnt-mediated-neuron-to-neuron-transfer-of-pathological-tau-protein-assemblies
#10
Meryem Tardivel, Séverine Bégard, Luc Bousset, Simon Dujardin, Audrey Coens, Ronald Melki, Luc Buée, Morvane Colin
A given cell makes exchanges with its neighbors through a variety of means ranging from diffusible factors to vesicles. Cells use also tunneling nanotubes (TNTs), filamentous-actin-containing membranous structures that bridge and connect cells. First described in immune cells, TNTs facilitate HIV-1 transfer and are found in various cell types, including neurons. We show that the microtubule-associated protein Tau, a key player in Alzheimer's disease, is a bona fide constituent of TNTs. This is important because Tau appears beside filamentous actin and myosin 10 as a specific marker of these fine protrusions of membranes and cytosol that are difficult to visualize...
November 4, 2016: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/27799074/activation-of-the-keap1-nrf2-stress-response-pathway-in-autophagic-vacuolar-myopathies
#11
Steve Duleh, Xianhong Wang, Allison Komirenko, Marta Margeta
Nrf2 (nuclear factor [erythroid-derived 2]-like 2; the transcriptional master regulator of the antioxidant stress response) is regulated through interaction with its cytoplasmic inhibitor Keap1 (Kelch-like ECH-associated protein 1), which under basal conditions targets Nrf2 for proteasomal degradation. Sequestosome 1 (SQSTM1)/p62-a multifunctional adapter protein that accumulates following autophagy inhibition and can serve as a diagnostic marker for human autophagic vacuolar myopathies (AVMs)-was recently shown to compete with Nrf2 for Keap1 binding, resulting in activation of the Nrf2 pathway...
October 31, 2016: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/27799073/nerve-pathologic-features-differentiate-poems-syndrome-from-cidp
#12
Ezequiel A Piccione, Janean Engelstad, Peter J Dyck, Michelle L Mauermann, Angela Dispenzieri, P James B Dyck
The objective of this study is to determine if the nerve pathology in patients with POEMS syndrome is different from CIDP. We hypothesized that nerve biopsies from patients with POEMS syndrome would have more small vessels and axonal degeneration but less inflammation than CIDP.We performed a retrospective analysis of nerve biopsies performed on "classic" CIDP and POEMS cases. Nerve biopsies were blinded and reviewed by two of the authors (EAP, PJBD). Teased fibers, paraffin-embedded sections, semithin sections and immunostains were analyzed...
October 31, 2016: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/27793194/activation-of-the-unfolded-protein-response-and-granulovacuolar-degeneration-are-not-common-features-of-human-prion-pathology
#13
Vera I Wiersma, Wim van Hecke, Wiep Scheper, Martijn A J van Osch, Will J M Hermsen, Annemieke J M Rozemuller, Jeroen J M Hoozemans
Human prion diseases are fatal neurodegenerative disorders with a genetic, sporadic or infectiously acquired aetiology. Neuropathologically, human prion diseases are characterized by deposition of misfolded prion protein and neuronal loss. In post-mortem brain tissue from patients with other neurodegenerative diseases characterized by protein misfolding, including Alzheimer's disease (AD) and frontotemporal lobar degeneration with tau pathology (FTLD-tau), increased activation of the unfolded protein response (UPR) has been observed...
October 28, 2016: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/27793193/hippocampal-phospho-tau-mapt-neuropathology-in-the-fornix-in-alzheimer-disease-an-immunohistochemical-autopsy-study
#14
Edward D Plowey, Jennifer L Ziskin
Whereas early Alzheimer disease (AD) neuropathology and mild cognitive impairment are relatively common in aging, accurate prediction of patients that will progress to dementia requires new biomarkers. Recently, substantial work has focused on phospho-tau/MAPT (p-MAPT) neuropathology since its regional propagation correlates with the degree of cognitive impairment in AD. Recent diffusion tensor imaging studies in AD suggest that increased diffusion in the fornix secondary to p-MAPT-related axonal injury could serve as a predictive biomarker of the risk of disease progression...
October 28, 2016: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/27793189/microglial-neuroinflammation-contributes-to-tau-accumulation-in-chronic-traumatic-encephalopathy
#15
Jonathan D Cherry, Yorghos Tripodis, Victor E Alvarez, Bertrand Huber, Patrick T Kiernan, Daniel H Daneshvar, Jesse Mez, Philip H Montenigro, Todd M Solomon, Michael L Alosco, Robert A Stern, Ann C McKee, Thor D Stein
The chronic effects of repetitive head impacts (RHI) on the development of neuroinflammation and its relationship to chronic traumatic encephalopathy (CTE) are unknown. Here we set out to determine the relationship between RHI exposure, neuroinflammation, and the development of hyperphosphorylated tau (ptau) pathology and dementia risk in CTE. We studied a cohort of 66 deceased American football athletes from the Boston University-Veteran's Affairs-Concussion Legacy Foundation Brain Bank as well as 16 non-athlete controls...
October 28, 2016: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/27788676/epidermal-growth-factor-prevents-apoe4-and-amyloid-beta-induced-cognitive-and-cerebrovascular-deficits-in-female-mice
#16
Riya Thomas, Paulina Zuchowska, Alan W J Morris, Felecia M Marottoli, Sangeeta Sunny, Ryan Deaton, Peter H Gann, Leon M Tai
Cerebrovascular (CV) dysfunction is emerging as a critical component of Alzheimer's disease (AD), including altered CV coverage. Angiogenic growth factors (AGFs) are key for controlling CV coverage, especially during disease pathology. Therefore, evaluating the effects of AGFs in vivo can provide important information on the role of CV coverage in AD. We recently demonstrated that epidermal growth factor (EGF) prevents amyloid-beta (Aβ)-induced damage to brain endothelial cells in vitro. Here, our goal was to assess the protective effects of EGF on cognition, CV coverage and Aβ levels using an AD-Tg model that incorporates CV relevant AD risk factors...
October 27, 2016: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/27724899/amyloid-%C3%AE-1-24-c-terminal-truncated-fragment-promotes-amyloid-%C3%AE-1-42-aggregate-formation-in-the-healthy-brain
#17
Sonia Mazzitelli, Fabia Filipello, Marco Rasile, Eliana Lauranzano, Chiara Starvaggi-Cucuzza, Matteo Tamborini, Davide Pozzi, Isabella Barajon, Toni Giorgino, Antonino Natalello, Michela Matteoli
Substantial data indicate that amyloid-β (Aβ), the major component of senile plaques, plays a central role in Alzheimer's Disease and indeed the assembly of naturally occurring amyloid peptides into cytotoxic aggregates is linked to the disease pathogenesis. Although Aβ42 is a highly aggregating form of Aβ, the co-occurrence of shorter Aβ peptides might affect the aggregation potential of the Aβ pool. In this study we aimed to assess whether the structural behavior of human Aβ42 peptide inside the brain is influenced by the concomitant presence of N-terminal fragments produced by the proteolytic activity of glial cells...
October 10, 2016: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/27717375/pglua%C3%AE-increases-accumulation-of-a%C3%AE-in-vivo-and-exacerbates-its-toxicity
#18
Oyinkan Sofola-Adesakin, Mobina Khericha, Inge Snoeren, Leo Tsuda, Linda Partridge
Several species of β-amyloid peptides (Aβ) exist as a result of differential cleavage from amyloid precursor protein (APP) to yield various C-terminal Aβ peptides. Several N-terminal modified Aβ peptides have also been identified in Alzheimer's disease (AD) brains, the most common of which is pyroglutamate-modified Aβ (AβpE3-42). AβpE3-42 peptide has an increased propensity to aggregate, appears to accumulate in the brain before the appearance of clinical symptoms of AD, and precedes Aβ1-42 deposition...
October 7, 2016: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/27716431/tor1a-mice-develop-dystonia-like-movements-via-a-striatal-dopaminergic-dysregulation-triggered-by-peripheral-nerve-injury
#19
Chi Wang Ip, Ioannis U Isaias, Burak B Kusche-Tekin, Dennis Klein, Janos Groh, Aet O'Leary, Susanne Knorr, Takahiro Higuchi, James B Koprich, Jonathan M Brotchie, Klaus V Toyka, Andreas Reif, Jens Volkmann
Isolated generalized dystonia is a central motor network disorder characterized by twisted movements or postures. The most frequent genetic cause is a GAG deletion in the Tor1a (DYT1) gene encoding torsinA with a reduced penetrance of 30-40 % suggesting additional genetic or environmental modifiers. Development of dystonia-like movements after a standardized peripheral nerve crush lesion in wild type (wt) and Tor1a+/- mice, that express 50 % torsinA only, was assessed by scoring of hindlimb movements during tail suspension, by rotarod testing and by computer-assisted gait analysis...
October 3, 2016: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/27716404/clinicopathological-characteristics-of-patients-with-amyotrophic-lateral-sclerosis-resulting-in-a-totally-locked-in-state-communication-stage-v
#20
Kentaro Hayashi, Yoko Mochizuki, Ryoko Takeuchi, Toshio Shimizu, Masahiro Nagao, Kazuhiko Watabe, Nobutaka Arai, Kiyomitsu Oyanagi, Osamu Onodera, Masaharu Hayashi, Hitoshi Takahashi, Akiyoshi Kakita, Eiji Isozaki
In the present study, we performed a comprehensive analysis to clarify the clinicopathological characteristics of patients with amyotrophic lateral sclerosis (ALS) that had progressed to result in a totally locked-in state (communication Stage V), in which all voluntary movements are lost and communication is impossible. In 11 patients, six had phosphorylated TAR DNA-binding protein 43 (pTDP-43)-immunoreactive (ir) neuronal cytoplasmic inclusions (NCI), two had fused in sarcoma (FUS)-ir NCI, and three had copper/zinc superoxide dismutase (SOD1)-ir NCI...
September 30, 2016: Acta Neuropathologica Communications
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