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Disease Models & Mechanisms

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https://www.readbyqxmd.com/read/28069688/up-regulation-of-cb2-receptors-in-reactive-astrocytes-in-canine-degenerative-myelopathy-a-disease-model-of-amyotrophic-lateral-sclerosis
#1
María Fernández-Trapero, Francisco Espejo-Porras, Carmen Rodríguez-Cueto, Joan R Coates, Carmen Pérez-Díaz, Eva de Lago, Javier Fernández-Ruiz
Targeting the CB2 receptor afforded neuroprotection in SOD1(G93A) mutant mice, a model of amyotrophic lateral sclerosis (ALS). The neuroprotective effects of CB2 receptors were facilitated by their up-regulation in the spinal cord in SOD1(G93A) mutant mice. Herein, we have investigated whether a similar CB2 receptor up-regulation, as well as parallel changes in other endocannabinoid elements, are evident in the spinal cord of dogs with degenerative myelopathy (DM), caused from mutations in the superoxide dismutase 1 gene (SOD1)...
January 9, 2017: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/28067631/schwann-cells-are-activated-by-atp-released-from-neurons-in-an-in-vitro-cellular-model-of-miller-fisher-syndrome
#2
Umberto Rodella, Samuele Negro, Michele Scorzeto, Elisanna Bergamin, Kees Jalink, Cesare Montecucco, Nobuhiro Yuki, Michela Rigoni
The neuromuscular junction is exposed to different types of insults including mechanical traumas, toxins or autoimmune antibodies and, accordingly, has retained through evolution a remarkable ability to regenerate. Regeneration is driven by multiple signals that are exchanged among the cellular components of the junction. These signals are largely unknown.Miller Fisher syndrome is a variant of Guillain-Barré syndrome caused by autoimmune antibodies specific for epitopes of peripheral axon terminals. Using an animal model of Miller Fisher syndrome, we recently reported that a monoclonal anti-polysialoganglioside GQ1b antibody plus complement damages nerve terminals with production of mitochondrial hydrogen peroxide, that activates Schwann cells...
January 6, 2017: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/28067630/novel-swine-model-of-ricin-induced-acute-respiratory-distress-syndrome
#3
Shahaf Katalan, Reut Falach, Amir Rosner, Michael Goldvaser, Tal Brosh-Nissimov, Ayana Dvir, Avi Mizrachi, Orr Goren, Barak Cohen, Yoav Gal, Anita Sapoznikov, Sharon Ehrlich, Tamar Sabo, Chanoch Kronman
Pulmonary exposure to the plant toxin ricin, leads to respiratory insufficiency and death. To date, in-depth study of the functional disorders ensuing pulmonary intoxication, a prerequisite for establishing a clinically-relevant therapeutic protocol, is hampered by the lack of an appropriate animal model. To this end, we set up the pig, as a large animal model for the comprehensive study of the multifarious clinical manifestations of pulmonary ricinosis.Here we report for the first time, the monitoring of barometric whole body plethysmography for pulmonary function tests in non-anesthetized ricin-intoxicated pigs...
January 6, 2017: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/28067625/pituitary-adenylate-cyclase-activating-polypeptide-pacap-has-neuroprotective-function-in-dopamine-based-neurodegeneration-developed-in-two-parkinsonian-models
#4
G Maasz, Z Zrinyi, D Reglodi, D Petrovics, A Rivnyak, T Kiss, A Jungling, A Tamas, Z Pirger
It has been observed that pituitary-adenylate cyclase activating polypeptide (PACAP) rescued DAergic neurons from neurodegeneration and improved motor alterations induced by 6-hydroxy-dopamine (6-OHDA) in rat parkinsonian models. Recently we investigated the molecular background of the neuroprotective effect of PACAP in DA-based neurodegeneration using rotenone-induced snail and 6-OHDA-induced rat models of Parkinson's disease. The behavioural activity, monoamine (DA and serotonin), metabolic enzyme (S-COMT, MB-COMT and MAO-B) and PARK7/DJ-1 protein contents were measured before and after PACAP-treatment in both models...
December 22, 2016: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/28067622/a-homozygous-fitm2-mutation-causes-a-deafness-dystonia-syndrome-with-motor-regression-and-signs-of-ichthyosis-and-sensory-neuropathy
#5
Celia Zazo Seco, Anna Castells-Nobau, Seol-Hee Joo, Margit Schraders, Jia Nee Foo, Monique van der Voet, S Sendhil Velan, Bonnie Nijhof, Jaap Oostrik, Erik de Vrieze, Radoslaw Katana, Atika Mansoor, Martijn Huynen, Radek Szklarczyk, Martin Oti, Lisbeth Tranebjærg, Erwin van Wijk, Jolanda M Scheffer-de Gooyert, Saadat Siddique, Jonathan Baets, Peter de Jonghe, Syed Ali Raza Kazmi, Suresh Anand Sadananthan, Bart P van de Warrenburg, Chiea Chuen Khor, Martin C Göpfert, Raheel Qamar, Annette Schenck, Hannie Kremer, Saima Siddiqi
A consanguineous family from Pakistan was ascertained with a novel deafness-dystonia syndrome with motor regression, ichthyosis-like features and signs of sensory neuropathy. By applying a combined strategy of linkage analysis and whole-exome sequencing in the presented family, a homozygous nonsense mutation, c.4G>T (p.Glu2*), in FITM2 was identified. FITM2 and its paralog FITM1 constitute an evolutionary conserved protein family involved in partitioning of triglycerides into cellular lipid droplets. Despite the role of FITM2 in neutral lipid storage and metabolism, no indications for lipodystrophy were observed in the affected individuals...
December 15, 2016: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/28067626/broad-aox-expression-in-a-genetically-tractable-mouse-model-does-not-disturb-normal-physiology
#6
Marten Szibor, Praveen K Dhandapani, Eric Dufour, Kira M Holmström, Yuan Zhuang, Isabelle Salwig, Ilka Wittig, Juliana Heidler, Zemfira Gizatullina, Timur Gainutdinov, German Mouse Clinic Consortium, Helmut Fuchs, Valérie Gailus-Durner, Martin Hrabě de Angelis, Jatin Nandania, Vidya Velagapudi, Astrid Wietelmann, Pierre Rustin, Frank N Gellerich, Howard T Jacobs, Thomas Braun
Plants and many lower organisms, but not mammals, express alternative oxidases (AOX) that branch the mitochondrial respiratory chain, transferring electrons directly from ubiquinol to oxygen without proton pumping. Thus, they maintain electron flow under conditions when the classical respiratory chain is impaired, limiting excess production of oxygen radicals and supporting redox and metabolic homeostasis. AOX from Ciona intestinalis has been used to study and mitigate mitochondrial impairments in mammalian cell-lines, Drosophila disease models and, most recently, in the mouse, where multiple, lentivector-AOX transgenes conferred substantial expression in specific tissues...
December 14, 2016: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/28067624/modelling-bronchopulmonary-dysplasia-in-mice-how-much-oxygen-is-enough
#7
Claudio Nardiello, Ivana Mižíková, Diogo M Silva, Jordi Ruiz-Camp, Konstantin Mayer, István Vadász, Susanne Herold, Werner Seeger, Rory E Morty
Progress in developing new therapies for bronchopulmonary dysplasia (BPD) is sometimes complicated by the lack of a standardised animal model. Our objective was to develop a robust hyperoxia‑based mouse model of BPD that recapitulated the pathological perturbations to lung structure noted in infants with BPD. Newborn mouse pups were exposed to a varying fraction of oxygen in the inspired air (FiO2) and a varying window of hyperoxia exposure, after which lung structure was assessed by design‑based stereology with systemic uniform random sampling...
December 14, 2016: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/28067623/seizure-control-through-genetic-and-pharmacological-manipulation-of-pumilio-a-key-component-of-neuronal-homeostasis
#8
Wei-Hsiang Lin, Carlo N G Giachello, Richard A Baines
Epilepsy is a significant disorder for which approximately one-third of patients do not respond to drug treatments. Next-generation drugs, which interact with novel targets, are required to provide a better clinical outcome for these individuals. To identify potential novel targets for antiepileptic drug (AED) design, we used RNA sequencing to identify changes in gene transcription in two seizure models of the fruitfly Drosophila melanogaster The first model compared gene transcription between wildtype (WT) and the bangsenseless(1) (para(bss)) mutant; a gain-of-function in the sole fly voltage-gated sodium channel (paralytic)...
December 14, 2016: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/27935821/stromal-hedgehog-signaling-maintains-smooth-muscle-and-hampers-micro-invasive-prostate-cancer
#9
Zhaohui Yang, Yu-Ching Peng, Anuradha Gopalan, Dong Gao, Yu Chen, Alexandra L Joyner
It is widely appreciated that reactive stroma or carcinoma-associated fibroblasts can influence epithelial tumor progression. In prostate cancer (PCa), the second most common male malignancy worldwide, the amount of reactive stroma is variable and has predictive value for tumor recurrence. By analyzing human PCa protein and RNA expression databases, we found smooth muscle cells (SMCs) are decreased in advanced tumors, whereas fibroblasts are maintained. In three mouse models of PCa, we found the composition of the stroma is distinct...
November 30, 2016: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/27935820/s113r-mutation-in-slc33a1-leads-to-neurodegeneration-and-augmented-bmp-signaling-in-a-mouse-model
#10
Pingting Liu, Baichun Jiang, Jian Ma, Pengfei Lin, Yinshuai Zhang, Changshun Shao, Wenjie Sun, Yaoqin Gong
The S113R mutation (c.339T>G) (MIM #603690.0001) in SLC33A1 (MIM #603690), an ER membrane acetyl-CoA transporter, has been previously identified in patients with hereditary spastic paraplegia type 42 (SPG42; MIM #612539). SLC33A1 has also been shown to inhibit bone morphogenetic protein (BMP) signaling pathway in zebrafish. To better understand the function of SLC33A1, we generated and characterized Slc33a1(S113R) knock-in mice. Homozygous Slc33a1(S113R) mutant mice were embryonic lethal, while heterozygous Slc33a1 mutant mice (Slc33a1(wt/mut)) exhibited behavioral abnormalities and central neurodegeneration, which is consistent with HSP phenotypes...
November 24, 2016: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/27935819/a-novel-brain-tumour-model-in-zebrafish-reveals-the-role-of-yap-activation-in-mapk-pi3k-induced-malignant-growth
#11
Marie Mayrhofer, Victor Gourain, Markus Reischl, Pierre Affaticati, Arnim Jenett, Jean-Stephane Joly, Matteo Benelli, Francesca Demichelis, Pietro Luigi Poliani, Dirk Sieger, Marina Mione
Somatic mutations activating MAPK/PI3K signalling play a pivotal role in both tumours and brain developmental disorders. We developed a zebrafish model of brain tumour based on somatic expression of oncogenes that activate MAPK/PI3K signalling in neural progenitor cells. HRAS(V12) was the most effective in inducing both heterotopia and invasive tumours. Tumours, but not heterotopias, require persistent activation of phospho‑(p)ERK and express a gene signature similar to the mesenchymal glioblastoma subtype, with a strong YAP component...
November 24, 2016: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/27935818/prenatal-ethanol-exposure-phenocopies-cdon-mutation-by-impeding-shh-function-in-the-etiology-of-optic-nerve-hypoplasia
#12
Benjamin M Kahn, Tanya S Corman, Korah Lovelace, Mingi Hong, Robert S Krauss, Douglas J Epstein
Septo-optic dysplasia (SOD) is a congenital disorder characterized by optic nerve, pituitary and midline brain malformations. The clinical presentation of SOD is highly variable with a poorly understood etiology. The majority of SOD cases are sporadic, but in rare instances inherited mutations have been identified in a small number of transcription factors, some of which regulate the expression of Sonic hedgehog (Shh) during mouse forebrain development. SOD is also associated with young maternal age suggesting that environmental factors, including alcohol consumption at early stages of pregnancy, may increase the risk of developing this condition...
November 24, 2016: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/28067629/standardized-echocardiographic-assessment-of-cardiac-function-in-normal-adult-zebrafish-and-heart-disease-models
#13
Louis W Wang, Inken G Huttner, Celine F Santiago, Scott H Kesteven, Ze-Yan Yu, Michael P Feneley, Diane Fatkin
The zebrafish (Danio rerio) is an increasingly popular model organism in cardiovascular research. Major insights into cardiac developmental processes have been gained by studies of embryonic zebrafish. However, the utility of zebrafish for modeling adult-onset heart disease has been limited by a lack of robust methods for in vivo evaluation of cardiac function. We established a physiological protocol for underwater zebrafish echocardiography using high frequency ultrasound, and evaluated its reliability in detecting altered cardiac function in two disease models...
January 1, 2017: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/28067628/3d-bioprinting-improving-in-vitro-models-of-metastasis-with-heterogeneous-tumor-microenvironments
#14
REVIEW
Jacob L Albritton, Jordan S Miller
Even with many advances in treatment over the past decades, cancer still remains a leading cause of death worldwide. Despite the recognized relationship between metastasis and increased mortality rate, surprisingly little is known about the exact mechanism of metastatic progression. Currently available in vitro models cannot replicate the three-dimensionality and heterogeneity of the tumor microenvironment sufficiently to recapitulate many of the known characteristics of tumors in vivo Our understanding of metastatic progression would thus be boosted by the development of in vitro models that could more completely capture the salient features of cancer biology...
January 1, 2017: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/28067627/susan-lindquist-a-tribute
#15
Vivian Siegel
No abstract text is available yet for this article.
January 1, 2017: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/27935825/microglia-activation-in-a-pediatric-rabbit-model-of-tuberculous-meningitis
#16
Elizabeth W Tucker, Supriya Pokkali, Zhi Zhang, Vincent P DeMarco, Mariah Klunk, Elizabeth S Smith, Alvaro A Ordonez, Marie-France Penet, Zaver Bhujwalla, Sanjay K Jain, Sujatha Kannan
Central nervous system (CNS) tuberculosis (TB) is the most severe form of extra-pulmonary TB and disproportionately affects young children where the developing brain has a unique host response. New Zealand white rabbits were infected with Mycobacterium tuberculosis via subarachnoid inoculation at postnatal day 4-8 and evaluated until 4-6 weeks post-infection. Control and infected rabbit kits were assessed for the development of neurological deficits, bacterial burden, and postmortem microbiologic and pathologic changes...
December 1, 2016: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/27935824/helicobacter-pylori-vaca-acting-through-receptor-protein-tyrosine-phosphatase-%C3%AE-is-crucial-for-caga-phosphorylation-in-human-duodenum-carcinoma-cell-line-az-521
#17
Masayuki Nakano, Kinnosuke Yahiro, Eiki Yamasaki, Hisao Kurazono, Junko Akada, Yoshio Yamaoka, Takuro Niidome, Masanori Hatakeyama, Hidekazu Suzuki, Taro Yamamoto, Joel Moss, Hajime Isomoto, Toshiya Hirayama
Helicobacter pylori, a major cause of gastroduodenal diseases, produces vacuolating cytotoxin (VacA) and cytotoxin-associated gene A (CagA), which seem to be involved in virulence. VacA exhibits pleiotropic actions in gastroduodenal disorders via its specific receptors. Recently, we found that VacA induced the phosphorylation of cellular Src kinase (Src) at Tyr418 in AZ-521 cells. Silencing of receptor protein tyrosine phosphatase (RPTP)α, a VacA receptor, reduced VacA-induced Src phosphorylation. Src is responsible for tyrosine phosphorylation of CagA at its Glu-Pro-Ile-Tyr-Ala (EPIYA) variant C (EPIYA-C) motif in Helicobacter pylori-infected gastric epithelial cells, resulting in binding of CagA to SHP-2 phosphatase...
December 1, 2016: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/27935823/renal-disease-pathophysiology-and-treatment-contributions-from-the-rat
#18
REVIEW
Linda J Mullins, Bryan R Conway, Robert I Menzies, Laura Denby, John J Mullins
The rat has classically been the species of choice for pharmacological studies and disease modeling, providing a source of high-quality physiological data on cardiovascular and renal pathophysiology over many decades. Recent developments in genome engineering now allow us to capitalize on the wealth of knowledge acquired over the last century. Here, we review rat models of hypertension, diabetic nephropathy, and acute and chronic kidney disease. These models have made important contributions to our understanding of renal diseases and have revealed key genes, such as Ace and P2rx7, involved in renal pathogenic processes...
December 1, 2016: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/27874836/whole-genome-sequence-snp-chips-and-pedigree-structure-building-demographic-profiles-in-domestic-dog-breeds-to-optimize-genetic-trait-mapping
#19
Dayna L Dreger, Maud Rimbault, Brian W Davis, Adrienne Bhatnagar, Heidi G Parker, Elaine A Ostrander
In the decade following publication of the draft genome sequence of the domestic dog, extraordinary advances with application to several fields have been credited to the canine genetic system. Taking advantage of closed breeding populations and the subsequent selection for aesthetic and behavioral characteristics, researchers have leveraged the dog as an effective natural model for the study of complex traits, such as disease susceptibility, behavior and morphology, generating unique contributions to human health and biology...
December 1, 2016: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/27874835/heterotypic-mouse-models-of-canine-osteosarcoma-recapitulate-tumor-heterogeneity-and-biological-behavior
#20
Milcah C Scott, Hirotaka Tomiyasu, John R Garbe, Ingrid Cornax, Clarissa Amaya, M Gerard O'Sullivan, Subbaya Subramanian, Brad A Bryan, Jaime F Modiano
Osteosarcoma (OS) is a heterogeneous and rare disease with a disproportionate impact because it mainly affects children and adolescents. Lamentably, more than half of patients with OS succumb to metastatic disease. Clarification of the etiology of the disease, development of better strategies to manage progression, and methods to guide personalized treatments are among the unmet health needs for OS patients. Progress in managing the disease has been hindered by the extreme heterogeneity of OS; thus, better models that accurately recapitulate the natural heterogeneity of the disease are needed...
December 1, 2016: Disease Models & Mechanisms
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