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https://www.readbyqxmd.com/read/28335041/the-cerebellum-and-its-wrapping-meninge-developmental-interplay-between-two-major-structures
#1
Martin Catala
Meninges have long been considered as a protective and supportive tissue for the central nervous system. Nevertheless, new developmental roles are now attributed to them. The meninges that surround the cerebellum come from the cephalic mesoderm. They are essential for the cerebellum to develop normally. They induce and maintain the basal lamina and glia limitans. In the absence of these structures, the external granular cells of the cerebellum migrate aberrantly and penetrate the subarachnoid space. The molecules involved in the recognition between the cerebellar primordium and the basal lamina belong to two groups in humans: dystroglycan and laminin on the one hand, and GPR56 and collagen III on the other...
March 23, 2017: Neuropediatrics
https://www.readbyqxmd.com/read/28335005/tdp-43-suppresses-tau-expression-via-promoting-its-mrna-instability
#2
Jianlan Gu, Feng Wu, Wen Xu, Jianhua Shi, Wen Hu, Nana Jin, Wei Qian, Xinglong Wang, Khalid Iqbal, Cheng-Xin Gong, Fei Liu
In the brains of individuals with Alzheimer's disease (AD) and chronic traumatic encephalopathy, tau pathology is accompanied usually by intracellular aggregation of transactive response DNA-binding protein 43 (TDP-43). However, the role of TDP-43 in tau pathogenesis is not understood. Here, we investigated the role of TDP-43 in tau expression in vitro and in vivo. We found that TDP-43 suppressed tau expression by promoting its mRNA instability through the UG repeats of its 3΄-untranslated region (3΄-UTR)...
March 15, 2017: Nucleic Acids Research
https://www.readbyqxmd.com/read/28334855/slc30a9-mutation-affecting-intracellular-zinc-homeostasis-causes-a-novel-cerebro-renal-syndrome
#3
Yonatan Perez, Zamir Shorer, Keren Liani-Leibson, Pauline Chabosseau, Rotem Kadir, Michael Volodarsky, Daniel Halperin, Shiran Barber-Zucker, Hanna Shalev, Ruth Schreiber, Libe Gradstein, Evgenia Gurevich, Raz Zarivach, Guy A Rutter, Daniel Landau, Ohad S Birk
A novel autosomal recessive cerebro-renal syndrome was identified in consanguineous Bedouin kindred: neurological deterioration was evident as of early age, progressing into severe intellectual disability, profound ataxia, camptocormia and oculomotor apraxia. Brain MRI was normal. Four of the six affected individuals also had early-onset nephropathy with features of tubulo-interstitial nephritis, hypertension and tendency for hyperkalemia, though none had rapid deterioration of renal function. Genome wide linkage analysis identified an ∼18 Mb disease-associated locus on chromosome 4 (maximal logarithm of odds score 4...
February 9, 2017: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/28334252/individualized-prediction-of-reading-comprehension-ability-using-gray-matter-volume
#4
Zaixu Cui, Mengmeng Su, Liangjie Li, Hua Shu, Gaolang Gong
Reading comprehension is a crucial reading skill for learning and putatively contains 2 key components: reading decoding and linguistic comprehension. Current understanding of the neural mechanism underlying these reading comprehension components is lacking, and whether and how neuroanatomical features can be used to predict these 2 skills remain largely unexplored. In the present study, we analyzed a large sample from the Human Connectome Project (HCP) dataset and successfully built multivariate predictive models for these 2 skills using whole-brain gray matter volume features...
March 10, 2017: Cerebral Cortex
https://www.readbyqxmd.com/read/28333139/inhibition-of-autophagy-prevents-irradiation-induced-neural-stem-and-progenitor-cell-death-in-the-juvenile-mouse-brain
#5
Yafeng Wang, Kai Zhou, Tao Li, Yiran Xu, Cuicui Xie, Yanyan Sun, Yaodong Zhang, Juan Rodriguez, Klas Blomgren, Changlian Zhu
Radiotherapy is an effective tool in the treatment of malignant brain tumors. However, damage to brain stem and progenitor cells constitutes a major problem and is associated with long-term side effects. Autophagy has been shown to be involved in cell death, and the purpose of this study was to evaluate the effect of autophagy inhibition on neural stem and progenitor cell death in the juvenile brain. Ten-day-old selective Atg7 knockout (KO) mice and wild-type (WT) littermates were subjected to a single 6Gy dose of whole-brain irradiation...
March 23, 2017: Cell Death & Disease
https://www.readbyqxmd.com/read/28331115/spontaneous-multicentric-soft-tissue-sarcoma-in-a-captive-african-pygmy-hedgehog-atelerix-albiventris-case-report-and-literature-review
#6
Josué Díaz-Delgado, Roy Pool, Sharman Hoppes, Argine Cerezo, Óscar Quesada-Canales, George Stoica
This report describes the clinical, macroscopic, histopathological and immunohistological features of a spontaneous multicentric extraskeletal sarcoma in an adult male African hedgehog (Atelerix albiventris). It also provides a succinct up-to-date review on neoplasia in this species. On autopsy examination, main gross findings included a moderately demarcated cranial mass and a multilobulated, caudal intra-abdominal mass. The cranial mass had perforated the underlying temporal and occipital bones and had extended into the cranial vault and was compressing the surface of the cerebellum and cerebrum...
March 23, 2017: Journal of Veterinary Medical Science
https://www.readbyqxmd.com/read/28330765/hsp90-and-pcreb-alterations-are-linked-to-mancozeb-dependent-behavioral-and-neurodegenerative-effects-in-a-marine-teleost
#7
Merylin Zizza, Mariana Di Lorenzo, Vincenza Laforgia, Emilia Furia, Giovanni Sindona, Marcello Canonaco, Rosa Maria Facciolo
The pesticide mancozeb (mz) is recognized as a potent inducer of oxidative stress due to its ability to catalyze the production of reactive oxygen species plus inhibiting mitochondrial respiration thus becoming an environmental risk for neurodegenerative diseases. Despite numerous toxicological studies on mz have been directed to mammals, attention on marine fish is still lacking. Thus, it was our intention to evaluate neurobehavioral activities of ornate wrasses (Thalassoma pavo) exposed to 0.2mg/l of mz after a preliminary screening test (0...
March 19, 2017: Toxicology and Applied Pharmacology
https://www.readbyqxmd.com/read/28329845/spatiotemporal-progression-and-remission-of-lesions-in-the-rat-brain-following-acute-intoxication-with-diisopropylfluorophosphate
#8
Sílvia Sisó, Brad A Hobson, Danielle J Harvey, Donald A Bruun, Douglas J Rowland, Joel R Garbow, Pamela J Lein
Similar to organophosphate (OP) nerve agents, diisopropylfluorophosphate (DFP) rapidly and irreversibly inhibits acetylcholinesterase, leading to convulsions that can progress to status epilepticus (SE). However, in contrast to the OP nerve agents, the long-term consequences of DFP-induced SE are not well known. Thus, we characterized the spatiotemporal profile of neuropathology during the first two months following acute DFP intoxication. Adult, male Sprague Dawley rats administered pyridostigmine bromide (0...
March 7, 2017: Toxicological Sciences: An Official Journal of the Society of Toxicology
https://www.readbyqxmd.com/read/28328116/brain-morphology-in-children-with-nevoid-basal-cell-carcinoma-syndrome
#9
Tadashi Shiohama, Katsunori Fujii, Toshiyuki Miyashita, Hiromi Mizuochi, Hideki Uchikawa, Naoki Shimojo
Brain morphology is tightly regulated by diverse signaling pathways. Hedgehog signaling is a candidate pathway considered responsible for regulating brain morphology. Nevoid basal cell carcinoma syndrome (NBCCS), caused by a PTCH1 mutation in the hedgehog signaling pathway, occasionally exhibits macrocephaly and medulloblastoma. Although cerebellar enlargement occurs in ptch1 heterozygous-deficient mice, its impact on human brain development remains unknown. We investigated the brain morphological characteristics of children with NBCCS...
April 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28327626/altered-resting-state-hippocampal-functional-networks-associated-with-chemotherapy-induced-prospective-memory-impairment-in-breast-cancer-survivors
#10
Huaidong Cheng, Wen Li, Liang Gong, Han Xuan, Zhonglian Huang, Hong Zhao, Long Sheng Wang, Kai Wang
In this study, we aimed to investigate the intrinsic hippocampal functional connectivity (FC) network and its relationship with prospective memory in patients with breast cancer suffering from chemotherapy-induced cognitive impairment (CICI). Thirty-four breast cancer patients before and after adjuvant chemotherapy (CB and CC, respectively) and 31 age- and education-matched cognitively normal (CN) women were recruited and subjected to a prospective memory task and a resting-state functional magnetic resonance imaging scan...
March 22, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28326436/reference-region-selection-and-the-association-between-the-rate-of-amyloid-accumulation-over-time-and-the-baseline-amyloid-burden
#11
Janusch Blautzik, Matthias Brendel, Julia Sauerbeck, Sebastian Kotz, Franziska Scheiwein, Peter Bartenstein, John Seibyl, Axel Rominger
Relative quantitative analysis of amyloid plaque burden in Alzheimer's disease (AD) patients can be reported as standardized uptake value ratio (SUVR) from positron emission tomography (PET). Here, the SUVR is the ratio of the mean amyloid radioligand retention in a composite (COMP) neocortical volume of interest (VOI) to that in a reference VOI, such as the cerebellum, brainstem (BST)/pons, or white matter (WM). Some longitudinal PET investigations show that the rate of amyloid accumulation to follow-up has an inverted U relationship with baseline amyloid SUVR relative to cerebellar or brainstem/pons reference VOIs...
March 22, 2017: European Journal of Nuclear Medicine and Molecular Imaging
https://www.readbyqxmd.com/read/28325680/prefrontal-activity-and-impaired-memory-encoding-strategies-in-schizophrenia
#12
Synthia Guimond, Colin Hawco, Martin Lepage
Schizophrenia patients have significant memory difficulties that have far-reaching implications in their daily life. These impairments are partly attributed to an inability to self-initiate effective memory encoding strategies, but its core neurobiological correlates remain unknown. The current study addresses this critical gap in our knowledge of episodic memory impairments in schizophrenia. Schizophrenia patients (n = 35) and healthy controls (n = 23) underwent a Semantic Encoding Memory Task (SEMT) during an fMRI scan...
March 8, 2017: Journal of Psychiatric Research
https://www.readbyqxmd.com/read/28323825/association-between-fatty-acid-metabolism-in-the-brain-and-alzheimer-disease-neuropathology-and-cognitive-performance-a-nontargeted-metabolomic-study
#13
Stuart G Snowden, Amera A Ebshiana, Abdul Hye, Yang An, Olga Pletnikova, Richard O'Brien, John Troncoso, Cristina Legido-Quigley, Madhav Thambisetty
BACKGROUND: The metabolic basis of Alzheimer disease (AD) pathology and expression of AD symptoms is poorly understood. Omega-3 and -6 fatty acids have previously been linked to both protective and pathogenic effects in AD. However, to date little is known about how the abundance of these species is affected by differing levels of disease pathology in the brain. METHODS AND FINDINGS: We performed metabolic profiling on brain tissue samples from 43 individuals ranging in age from 57 to 95 y old who were stratified into three groups: AD (N = 14), controls (N = 14) and "asymptomatic Alzheimer's disease" (ASYMAD), i...
March 2017: PLoS Medicine
https://www.readbyqxmd.com/read/28323163/cerebrospinal-and-interstitial-fluid-transport-via-the-glymphatic-pathway-modeled-by-optimal-mass-transport
#14
Vadim Ratner, Yi Gao, Hedok Lee, Rena Elkin, Maiken Nedergaard, Helene Benveniste, Allen Tannenbaum
The glymphatic pathway is a system which facilitates continuous cerebrospinal fluid (CSF) and interstitial fluid (ISF) exchange and plays a key role in removing waste products from the rodent brain. Dysfunction of the glymphatic pathway may be implicated in the pathophysiology of Alzheimer's disease. Intriguingly, the glymphatic system is most active during deep wave sleep general anesthesia. By using paramagnetic tracers administered into CSF of rodents, we previously showed the utility of MRI in characterizing a macroscopic whole brain view of glymphatic transport but we have yet to define and visualize the specific flow patterns...
March 16, 2017: NeuroImage
https://www.readbyqxmd.com/read/28322325/a-non-cell-autonomous-mouse-model-of-cns-haemangioblastoma-mediated-by-mutant-kras
#15
Leyuan Bao, Osama Al-Assar, Lesley F Drynan, Mark J Arends, Pam Tyers, Roger A Barker, Terence H Rabbitts
Haemangioblastoma is a rare malignancy of the CNS where vascular proliferation causes lesions due to endothelial propagation. We found that conditionally expressing mutant Kras, using Rag1-Cre, gave rise to CNS haemangioblastoma in the cortex and cerebellum in mice that present with highly vascular tumours with stromal cells similar to human haemangioblastomas. The aberrant haemangioblastoma endothelial cells do not express mutant Kras but rather the mutant oncogene is expressed in CNS interstitial cells, including neuronal cells and progeny...
March 21, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28322272/neuroanatomic-epigenetic-and-genetic-differences-in-monozygotic-twins-discordant-for-attention-deficit-hyperactivity-disorder
#16
Y-C Chen, G Sudre, W Sharp, F Donovan, S C Chandrasekharappa, N Hansen, L Elnitski, P Shaw
The study of monozygotic twins discordant for attention deficit hyperactivity disorder can elucidate mechanisms that contribute to the disorder, which affects ~7% of children. First, using in vivo neuroanatomic imaging on 14 pairs of monozygotic twins (mean age 9.7, s.d. 1.9 years), we found that discordance for the disorder is mirrored by differing dimensions of deep brain structures (the striatum and cerebellum), but not the cerebral cortex. Next, using whole-blood DNA from the same twins, we found a significant enrichment of epigenetic differences in genes expressed in these 'discordant' brain structures...
March 21, 2017: Molecular Psychiatry
https://www.readbyqxmd.com/read/28321950/the-cerebellum-shrinks-faster-than-normal-ageing-in-alzheimer-s-disease-but-not-in-mild-cognitive-impairment
#17
Hossein Tabatabaei-Jafari, Erin Walsh, Marnie E Shaw, Nicolas Cherbuin
BACKGROUND: While acceleration in age-related cerebral atrophy has been well documented in Alzheimer's disease, the cerebellar contributions to this effect have not been thoroughly investigated. OBJECTIVE: This study investigated cerebellar volume and atrophy rate using magnetic resonance imaging in individuals with normal cognition (CN), mild cognitive impairment (MCI), and Alzheimer's disease (AD). METHODS: Two hundred twenty-nine CN, 398 MCI and 191 AD participants of stage I ADNI database with screening scans were evaluated for cerebellar volume...
March 21, 2017: Human Brain Mapping
https://www.readbyqxmd.com/read/28321713/low-titre-gad-antibody-associated-late-onset-cerebellar-ataxia-with-a-significant-clinical-response-to-intravenous-immunoglobulin-treatment
#18
Timotej Petrijan, Marija Menih
Antiglutamic acid decarboxylase antibody-associated cerebellar ataxia (GAD-Abs CA) is a rare, but increasingly detected, autoimmune neurological disorder characterized by the clinical presence of a cerebellar syndrome concomitant with positive GAD-Abs levels in serum and cerebrospinal fluid (CSF). It represents 3% of all immune-mediated sporadic CAs. Low-titre GAD-Abs CA is an even rarer subtype of GAD-Abs CA. We report on a 68-year-old woman with a 3-year history of progressive gait ataxia. In addition to the modified Rankin Scale (mRS), we used two other objective scales to evaluate CA severity, i...
March 20, 2017: Cerebellum
https://www.readbyqxmd.com/read/28321129/cerebellar-granule-cells-encode-the-expectation-of-reward
#19
Mark J Wagner, Tony Hyun Kim, Joan Savall, Mark J Schnitzer, Liqun Luo
The human brain contains approximately 60 billion cerebellar granule cells, which outnumber all other brain neurons combined. Classical theories posit that a large, diverse population of granule cells allows for highly detailed representations of sensorimotor context, enabling downstream Purkinje cells to sense fine contextual changes. Although evidence suggests a role for the cerebellum in cognition, granule cells are known to encode only sensory and motor context. Here, using two-photon calcium imaging in behaving mice, we show that granule cells convey information about the expectation of reward...
March 20, 2017: Nature
https://www.readbyqxmd.com/read/28320419/cerebellar-ependymoma-with-overlapping-features-of-clear-cell-and-tanycytic-variants-mimicking-hemangioblastoma-a-case-report-and-literature-review
#20
Xiu-Peng Zhang, Yang Liu, Di Zhang, Qin Zheng, Chen Wang, Liang Wang, Qing-Chang Li, Xue-Shan Qiu, En-Hua Wang
BACKGROUND: Imaging and histology of clear-cell ependymoma and cerebellum-based hemangioblastoma are similar; distinguishing between them is a diagnostic challenge. CASE PRESENTATION: A 62-year-old Chinese woman presented with an intermittent headache of 8 years' duration. Computed tomography and magnetic resonance imaging revealed a mass in the cerebellum. Neurological imaging suggested hemangioblastoma (HB). Histologically, the tumor included cellular and paucicellular areas, in which cells were arranged in nests or diffusely distributed; and a highly vascular area, in which tumor cells were arranged in clusters and separated by capillaries...
March 20, 2017: Diagnostic Pathology
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