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Disease Models & Mechanisms

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https://www.readbyqxmd.com/read/29752300/intramyocardial-angiogenetic-stem-cells-and-epicardial-erythropoietin-save-the-acute-ischemic-heart
#1
Christian Klopsch, Anna Skorska, Marion Ludwig, Heiko Lemcke, Gabriela Maass, Ralf Gaebel, Martin Beyer, Cornelia Lux, Anita Toelk, Karina Müller, Christian Maschmeier, Sarah Rohde, Petra Mela, Brigitte Müller-Hilke, Stefan Jockenhoevel, Brigitte Vollmar, Robert Jaster, Robert David, Gustav Steinhoff
Ischemic heart failure still displays the highest mortality. An early boost of intracardiac regenerative key mechanisms and angiogenetic niche signaling in cardiac mesenchymal stem cells (MSCs) could improve myocardial infarction (MI) healing. Epicardial erythropoietin (EPO, 300U kg-1 ) was compared with intraperitoneal and intramyocardial EPO treatments after acute MI in rats (n=156). Real-time PCR and confocal microscopy revealed epicardial EPO treatment enhanced intracardiac regenerative key indicators (SDF-1, CXCR-4, CD34, Bcl-2, Cyclin D1, Cdc2, MMP2), induced TGF-β/WNT signaling in intramyocardial MSC niches through direct activation of AKT, upregulations of upstream signals FOS and Fzd7 and augmented intracardiac mesenchymal proliferation 24 hours after MI...
May 10, 2018: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/29752287/-rnaset2-knockout-rats-exhibit-hippocampal-neuropathology-and-deficits-in-memory
#2
Kerstin W Sinkevicius, Thomas R Morrison, Praveen Kulkarni, Martha K Caffrey Cagliostro, Sade Iriah, Samantha Malmberg, Julia Sabrick, Jennifer A Honeycutt, Kim L Askew, Malav Trivedi, Craig F Ferris
RNASET2 deficiency in humans is associated with infant cystic leukoencephalopathy, which causes psychomotor impairment, spasticity, and epilepsy. A zebrafish mutant model suggests that loss of RNASET2 function leads to neurodegeneration due to the accumulation of non-degraded RNA in the lysosomes. The goal of this study was to characterize the first rodent model of RNASET2 deficiency. The brains of 3- and 12-month-old RNaseT2 knockout rats were studied using multiple magnetic resonance imaging modalities and behavioral tests...
May 10, 2018: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/29752286/genetic-and-cellular-sensitivity-of-caenorhabditis-elegans-to-the-chemotherapeutic-agent-cisplatin
#3
Francisco Javier García-Rodríguez, Carmen Martínez-Fernández, David Brena, Dmytro Kukhtar, Xènia Serrat, Ernest Nadal, Mike Boxem, Sebastian Honnen, Antonio Miranda-Vizuete, Alberto Villanueva, Julián Cerón
Cisplatin and derivatives are commonly used as chemotherapeutic agents. Although the cytotoxic action of cisplatin on cancer cells is very efficient, clinical oncologists need to deal with two major difficulties: (i) the onset of resistance to the drug, and (ii) the cytotoxic effect in patients. Here we use Caenorhabditis elegans to investigate factors influencing the response to cisplatin in multicellular organisms. In this hermaphroditic model organism, we observed that sperm failure is a major cause in cisplatin-induced infertility...
May 10, 2018: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/29752281/increased-fgf8-signaling-promotes-chondrogenic-rather-than-osteogenic-development-in-the-embryonic-skull
#4
Linnea Schmidt, Aftab Taiyab, Vida Senkus Melvin, Kenneth L Jones, Trevor Williams
The bones of the cranial vault are formed directly from mesenchymal cells through intramembranous ossification rather than via a cartilage intermediate. Formation and growth of the skull bones involves the interaction of multiple cell:cell signaling pathways, with Fibroblast Growth Factors (FGFs) and their receptors exerting prominent influence. Mutations within this pathway are the most frequent cause of craniosynostosis, which is a common human craniofacial developmental abnormality characterized by the premature fusion of the cranial sutures...
May 10, 2018: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/29752279/a-comprehensive-study-of-calcific-aortic-stenosis-from-rabbit-to-human-samples
#5
L Mourino-Alvarez, M Baldan-Martin, T Sastre-Oliva, M Martin-Lorenzo, A S Maroto, N Corbacho-Alonso, R Rincon, T Martin-Rojas, L F Lopez-Almodovar, G Alvarez-Llamas, F Vivanco, L R Padial, F de la Cuesta, M G Barderas
The global incidence of calcific aortic stenosis (CAS) is increasing due, in part, to a growing elderly population, and poses a great challenge to public health because of the multiple comorbidities of these patients. Using a rabbit model of CAS, we sought to characterize protein alterations associated with calcified valve tissue that can be ultimately measured in plasma as non-invasive biomarkers of CAS.Aortic valves from healthy and mild stenotic rabbits were analyzed by two-dimensional difference gel electrophoresis, and selected reaction monitoring was used to directly measure the differentially expressed proteins in plasma from the same rabbits to corroborate their potential as diagnostic indicators, and also in plasma from human subjects, to examine their translatability to the clinical setting...
May 10, 2018: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/29739816/-cacna1c-haploinsufficiency-leads-to-pro-social-50-khz-ultrasonic-communication-deficits-in-rats
#6
Theresa M Kisko, Moria D Braun, Susanne Michels, Stephanie H Witt, Marcella Rietschel, Carsten Culmsee, Rainer K W Schwarting, Markus Wöhr
The cross-disorder risk gene CACNA1C is strongly implicated in multiple neuropsychiatric disorders, including autism spectrum disorder (ASD), bipolar disorder (BPD), and schizophrenia (SCZ), with deficits in social functioning being common for all major neuropsychiatric disorders. In the present study, we explored the role of Cacna1c in regulating disorder-relevant behavioral phenotypes, focusing on socio-affective communication after weaning during the critical developmental period of adolescence in rats. To this aim, we used a newly developed genetic Cacna1c rat model and applied a truly reciprocal approach for studying communication through ultrasonic vocalizations, including both sender and receiver...
May 8, 2018: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/29739804/etiology-and-treatment-of-adrenoleukodystrophy-new-insights-from-drosophila
#7
Hannah B Gordon, Lourdes Valdez, Anthea Letsou
Adrenoleukodystrophy (ALD) is a fatal progressive neurodegenerative disorder affecting brain white matter. The most common form of ALD is X-linked (X-ALD) and results from mutation of the ABCD1 -encoded very long chain fatty acid (VLCFA) transporter. X-ALD is clinically heterogeneous, with the cerebral form being the most severe. Diagnosed in boys usually between the ages of 4 and 8, cerebral X-ALD symptoms progress rapidly (in as little as two years) through declines in cognition, learning, and behavior, to paralysis and ultimately to a vegetative state and death...
May 8, 2018: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/29728408/collagen-vi-in-healthy-and-diseased-nervous-system
#8
REVIEW
Ilaria Gregorio, Paola Braghetta, Paolo Bonaldo, Matilde Cescon
Collagen VI is a major extracellular matrix protein exerting a number of functions in different tissues, spanning from biomechanical to regulatory signals in the cell survival processes, and playing key roles in maintaining the stemness or determining the differentiation of several types of cells. In the last couple of years, emerging findings on collagen VI have led to increased interest in its role in the nervous system. The role of this protein in the peripheral nervous system was intensely studied and characterized in detail...
May 4, 2018: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/29728409/subchondral-drilling-for-articular-cartilage-repair-a-systematic-review-of-translational-research
#9
Liang Gao, Lars K H Goebel, Patrick Orth, Magali Cucchiarini, Henning Madry
Articular cartilage defects may initiate osteoarthritis. Subchondral drilling, a widely applied clinical technique to treat small cartilage defects, does not yield cartilage regeneration. Various translational studies aiming to improve the outcome of drilling have been performed, however, a robust systematic analysis of its translational evidence has been still lacking. Here, we performed a systematic review of the outcome of subchondral drilling for knee cartilage repair in translational animal models. A total of 12 relevant publications studying 198 animals were identified, detailed study characteristics were extracted, and methodological quality and risk of bias were analyzed...
May 3, 2018: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/29720474/functional-and-genomic-characterization-of-a-xenograft-model-system-for-the-study-of-metastasis-in-triple-negative-breast-cancer
#10
Cameron N Johnstone, Andrew D Pattison, Kylie L Gorringe, Paul F Harrison, David R Powell, Peter Lock, David Baloyan, M Ernst, Alastair G Stewart, Traude H Beilharz, Robin L Anderson
Triple-negative breast cancer represents 10-20% of all human ductal adenocarcinomas and has a poor prognosis relative to other subtypes. Hence, new molecular targets for therapeutic intervention are necessary. Analyses of panels of human or mouse cancer lines derived from the same individual that differ in their cellular phenotypes but not in genetic background have been instrumental in defining the molecular players that drive the various hallmarks of cancer.To determine the molecular regulators of metastasis in triple-negative breast cancer, we completed a rigorous in vitro and in vivo characterization of four populations of the MDA-MB-231 human breast cancer line ranging in aggressiveness from non-metastatic to spontaneously metastatic to lung, liver, spleen and lymph node...
April 30, 2018: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/29720471/bone-marrow-transplantation-corrects-haemolytic-anaemia-in-novel-enu-mutagenesis-mouse-model-of-tpi-deficiency
#11
Ashlee J Conway, Fiona C Brown, Elinor J Hortle, Gaetan Burgio, Simon J Foote, Craig J Morton, Stephen M Jane, David J Curtis
We have performed a genome-wide ENU mutagenesis screen in mice to identify novel genes or alleles that regulate erythropoiesis. Here we describe a recessive mouse strain, called RBC19, harbouring a point mutation within the housekeeping gene, Tpi1 , which encodes for the glycolysis enzyme, triosephosphate isomerase (TPI). A serine in place of a phenylalanine at amino acid 57 severely diminishes enzyme activity in red cells and other tissues, resulting in a macrocytic haemolytic phenotype in homozygous mice that closely resembles human TPI deficiency...
April 30, 2018: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/29716962/bruno-3-regulates-sarcomere-components-expression-and-contributes-to-muscle-phenotypes-of-myotonic-dystrophy-type-1
#12
L Picchio, V Legagneux, S Deschamps, Y Renaud, S Chauveau, L Paillard, K Jagla
Steinert disease or Myotonic dystrophy type 1 (DM1) is a multisystemic disorder caused by toxic non-coding CUG repeat transcripts leading to altered levels of two RNA binding factors, MBNL1 and CELF1. The contribution of CELF1 to DM1 phenotypes is controversial. Here, we show that Drosophila CELF1 family member Bru - 3, contributes to pathogenic muscle defects observed in Drosophila model of DM1. Bru-3 displays predominantly cytoplasmic expression in muscles and its muscle-specific overexpression causes a range of phenotypes also observed in fly DM1 model including affected motility, fiber splitting, reduced myofiber length and altered myoblast fusion...
April 30, 2018: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/29716957/lifespan-analysis-of-brain-development-gene-expression-and-behavioral-phenotypes-in-the-ts1cje-ts65dn-and-dp-16-1-yey-mouse-models-of-down-syndrome
#13
Nadine M Aziz, Faycal Guedj, Jeroen L A Pennings, Jose Luis Olmos-Serrano, Ashley Siegel, Tarik F Haydar, Diana W Bianchi
Down syndrome (DS) results from triplication of human chromosome 21. Neuropathological hallmarks of DS include atypical central nervous system development that manifests prenatally and extends throughout life. As a result, individuals with DS exhibit cognitive and motor deficits and have delays in achieving developmental milestones. To determine whether different mouse models of DS recapitulate the human prenatal and postnatal phenotypes, here we directly compared brain histogenesis, gene expression, and behavior over the lifespan of three cytogenetically distinct mouse models of DS: Ts1Cje, Ts65Dn and Dp(16)1/Yey...
April 30, 2018: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/29716954/nmnat-mitigates-sensory-dysfunction-in-a-drosophila-model-of-paclitaxel-induced-peripheral-neuropathy
#14
Jennifer M Brazill, Beverley Cruz, Yi Zhu, R Grace Zhai
Chemotherapy-induced peripheral neuropathy (CIPN) is the major dose-limiting side effect of many commonly used chemotherapeutic agents, including paclitaxel. Currently there are no neuroprotective or effective symptomatic treatments for CIPN. Lack of understanding of the in vivo mechanisms of CIPN has greatly impeded the identification of therapeutic targets. Here we optimized a model of paclitaxel-induced peripheral neuropathy using Drosophila larvae that recapitulates aspects of chemotherapy-induced sensory dysfunction ...
April 30, 2018: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/29716894/generation-of-an-animal-model-of-pten-hamartoma-tumour-syndrome-in-the-retina
#15
Nobuhiko Tachibana, Yacine Touahri, Rajiv Dixit, Luke Ajay David, Lata Adnani, Robert Cantrup, Tooka Aavani, Rachel O Wong, Cairine Logan, Kyle C Kurek, Carol Schuurmans
PTEN Hamartoma Tumor Syndrome (PHTS) is a heterogeneous group of rare, autosomal dominant disorders associated with PTEN germline mutations. PHTS patients routinely develop hamartomas, which are benign tissue overgrowths comprised of disorganized 'normal' cells. Efforts to generate PHTS animal models have been largely unsuccessful due to the early lethality of homozygous germline mutations in Pten , together with the lack of hamartoma formation in most conditional mutants generated to date. We report herein a novel PHTS mouse model that reproducibly forms hamartoma-like lesions in the central retina by postnatal day 21...
April 24, 2018: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/29666142/tumor-xenograft-modeling-identifies-tcf4-itf2-loss-associated-with-breast-cancer-chemoresistance
#16
Gorka Ruiz de Garibay, Francesca Mateo, Agostina Stradella, Rafael Valdés-Mas, Luis Palomero, Jordi Serra-Musach, Diana A Puente, Ander Díaz-Navarro, Gardenia Vargas-Parra, Eva Tornero, Idoia Morilla, Lourdes Farré, María Martinez-Iniesta, Carmen Herranz, Emmet McCormack, August Vidal, Anna Petit, Teresa Soler, Conxi Lázaro, Xose S Puente, Alberto Villanueva, Miguel Angel Pujana
Understanding the mechanisms of cancer therapeutic resistance is fundamental to improving cancer care. There is clear benefit from chemotherapy in different breast cancer settings; however, knowledge of the mutations and genes that mediate resistance is incomplete. In this study, by modeling chemoresistance in patient-derived xenografts (PDXs), we show that adaptation to therapy is genetically complex and identify loss of transcription factor 4 (TCF4) associated with this process. A triple-negative BRCA1 -mutated PDX was used to study the genetics of chemoresistance...
April 13, 2018: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/29666220/synergistic-anti-proliferative-effect-of-mtor-inhibitor-rad001-plus-gemcitabine-on-cholangiocarcinoma-by-decreasing-choline-kinase-activity
#17
Gigin Lin, Kun-Ju Lin, Frank Wang, Tse-Ching Chen, Tzu-Chen Yen, Ta-Sen Yeh
Although gemcitabine plus cisplatin is the gold standard chemotherapy regimen for advanced cholangiocarcinoma, the response rate has been disappointing. This study aims to investigate a novel therapeutic regimen (gemcitabine plus rad001, an mTOR inhibitor) for cholangiocarcinoma. Gemcitabine, oxaliplatin, cetuximab, and rad001 in various combinations were first evaluated in vitro using six cholangiocarcinoma cell lines. In vivo therapeutic efficacies of gemcitabine, rad001 alone and combination were further evaluated using a xenograft mouse model and a chemically induced orthotopic cholangiocarcinoma rat model...
April 12, 2018: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/29666155/sil1-the-er-hsp70-co-chaperone-plays-a-critical-role-in-maintaining-skeletal-muscle-proteostasis-and-physiology
#18
Viraj P Ichhaporia, Jieun Kim, Kanisha Kavdia, Peter Vogel, Linda Horner, Sharon Frase, Linda M Hendershot
Mutations in SIL1 , a co-factor for the endoplasmic reticular (ER) chaperone BiP, cause Marinesco-Sjögren syndrome (MSS), an autosomal recessive disorder. Using a mouse model, we characterized molecular aspects of the progressive myopathy associated with MSS. Proteomic-profiling of quadriceps at the onset of myopathy revealed that SIL1 deficiency affected multiple pathways critical to muscle physiology. We observed an increase in ER chaperones prior to the onset of muscle weakness, which was complemented by up-regulation of multiple components of cellular protein degradation pathways...
April 9, 2018: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/29622551/choroidal-pericytes-promote-subretinal-fibrosis-after-experimental-photocoagulation
#19
Xueting Luo, Shiqi Yang, Jian Liang, Yuanqi Zhai, Mengxi Shen, Junran Sun, Yiji Feng, Xinmin Lu, Hong Zhu, Fenghua Wang, Xiaodong Sun
Subretinal fibrosis results in local destruction of retinal structures and permanent vision loss, representing the end stage of neovascular age-related macular degeneration (AMD). Histological examination of fibrotic specimens from AMD patients has uncovered a wide range of cellular and acellular components. However, their origins and roles in fibrosis remain largely unexplored. Using laser-induced photocoagulation model with Collagen 1α1-GFP reporter mice, we demonstrate by cell-lineage tracing that a subset of pericytes associating with choroidal microvasculature are activated upon injury and infiltrate into subretinal space as significant components of fibrotic lesions...
April 4, 2018: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/29592890/a-novel-zebrafish-intestinal-tumor-model-reveals-a-role-for-cyp7a1-dependent-tumor-liver-crosstalk-in-tumor-s-adverse-effects-on-host
#20
Sora Enya, Koichi Kawakami, Yutaka Suzuki, Shinpei Kawaoka
The nature of host organs and genes that underlie tumor-induced physiological disruption on host remains ill-defined. Here, we establish a novel zebrafish intestinal tumor model that is suitable for addressing this issue, and find that hepatic cyp7a1 , the rate-limiting factor for synthesizing bile acids, in the case of zebrafish bile alcohol (BA), is such a host gene. Inducing kras G12D by Gal4 specifically expressed in the posterior intestine resulted in formation of intestinal tumor. The local intestinal tumor caused systemic detrimental effects on host including liver inflammation, hepatomegaly, growth defects, and organismal death...
March 28, 2018: Disease Models & Mechanisms
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