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  Open access

June, 2018; 11 (6)

EDITORIAL

  • Open Access
    Tracking progress: an update on animal models for Duchenne muscular dystrophy
    Dominic J. Wells
    Disease Models & Mechanisms 2018 11: dmm035774 doi: 10.1242/dmm.035774 Published 13 June 2018

    Summary: With the development of a novel rabbit model of Duchenne muscular dystrophy (dmm032201), this Editorial reflects on the current animal models of this disease, their promise, prospects and pitfalls.

REVIEWS

  • Open Access
    Using Drosophila to study mechanisms of hereditary hearing loss
    Tongchao Li, Hugo J. Bellen, Andrew K. Groves
    Disease Models & Mechanisms 2018 11: dmm031492 doi: 10.1242/dmm.031492 Published 31 May 2018

    Summary: This Review discusses the similarities and differences between hearing in mammals and the fruit fly Drosophila, and describes how recent technological developments allow Drosophila to be used as a model to understand the function of human deafness genes.

  • Open Access
    Collagen VI in healthy and diseased nervous system
    Ilaria Gregorio, Paola Braghetta, Paolo Bonaldo, Matilde Cescon
    Disease Models & Mechanisms 2018 11: dmm032946 doi: 10.1242/dmm.032946 Published 31 May 2018

    Summary: Although collagen VI was so far known to be mostly involved in skeletal muscle homeostasis, this Review summarizes a number of recent studies that highlight its key role in the peripheral and central nervous systems, pointing at a link with human neurological disorders.

RESEARCH ARTICLES

  • Open Access
    RNaseT2 knockout rats exhibit hippocampal neuropathology and deficits in memory
    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
    Disease Models & Mechanisms 2018 11: dmm032631 doi: 10.1242/dmm.032631 Published 27 June 2018

    Summary: The authors characterize a rodent model of RNaseT2 deficiency, which offers insight into the susceptibility of the hippocampus to early inflammation caused by lysosome impairment due to loss of RNaseT2 function.

  • Open Access
    Adding a temporal dimension to the study of Friedreich's ataxia: the effect of frataxin overexpression in a human cell model
    Tommaso Vannocci, Roberto Notario Manzano, Ombretta Beccalli, Barbara Bettegazzi, Fabio Grohovaz, Gianfelice Cinque, Antonio de Riso, Luca Quaroni, Franca Codazzi, Annalisa Pastore
    Disease Models & Mechanisms 2018 11: dmm032706 doi: 10.1242/dmm.032706 Published 25 June 2018

    Summary: We have probed the effect of overexpression of the frataxin gene in an inducible cellular model. Our data indicate that the levels of frataxin must be tightly regulated, because any imbalance leads to oxidative stress and toxicity.

  • Open Access
    Intramyocardial angiogenetic stem cells and epicardial erythropoietin save the acute ischemic heart
    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
    Disease Models & Mechanisms 2018 11: dmm033282 doi: 10.1242/dmm.033282 Published 22 June 2018

    Summary: Cardiac mesenchymal stem cells respond early to myocardial infarction. Their regenerative capacity is directly upregulated by optimized stimulation of the heart through epicardial erythropoietin delivery.

  • Open Access
    Genetic and cellular sensitivity of Caenorhabditis elegans to the chemotherapeutic agent cisplatin
    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
    Disease Models & Mechanisms 2018 11: dmm033506 doi: 10.1242/dmm.033506 Published 21 June 2018

    Summary: Caenorhabditis elegans is a valuable model to identify genetic factors influencing the animal response to the widely used chemotherapeutic agent cisplatin.

  • Open Access
    Cacna1c haploinsufficiency leads to pro-social 50-kHz ultrasonic communication deficits in rats
    Theresa M. Kisko, Moria D. Braun, Susanne Michels, Stephanie H. Witt, Marcella Rietschel, Carsten Culmsee, Rainer K. W. Schwarting, Markus Wöhr
    Disease Models & Mechanisms 2018 11: dmm034116 doi: 10.1242/dmm.034116 Published 20 June 2018

    Summary: The present study suggests that Cacna1c plays a prominent role in regulating socio-affective communication in rats with relevance for neuropsychiatric disorders.

  • Open Access
    Subchondral drilling for articular cartilage repair: a systematic review of translational research
    Liang Gao, Lars K. H. Goebel, Patrick Orth, Magali Cucchiarini, Henning Madry
    Disease Models & Mechanisms 2018 11: dmm034280 doi: 10.1242/dmm.034280 Published 19 June 2018

    Summary: Cartilage defects initiate osteoarthritis. This is the first systematic review of translational evidence of cartilage defects in animal models treated by drilling, enhancing translation from basic science to clinical application.

  • Open Access
    A comprehensive study of calcific aortic stenosis: from rabbit to human samples
    Laura Mourino-Alvarez, Montserrat Baldan-Martin, Tamara Sastre-Oliva, Marta Martin-Lorenzo, Aroa Sanz Maroto, Nerea Corbacho-Alonso, Raul Rincon, Tatiana Martin-Rojas, Luis Fernando Lopez-Almodovar, Gloria Alvarez-Llamas, Fernando Vivanco, Luis Rodriguez Padial, Fernando de la Cuesta, Maria Gonzalez Barderas
    Disease Models & Mechanisms 2018 11: dmm033423 doi: 10.1242/dmm.033423 Published 19 June 2018

    Summary: Using a rabbit model of calcific aortic stenosis, we have defined a molecular panel of three proteins related to osteoblastic differentiation. Additionally, this panel has been confirmed in human samples.

  • Open Access
    Epigenetic regulators Rbbp4 and Hdac1 are overexpressed in a zebrafish model of RB1 embryonal brain tumor, and are required for neural progenitor survival and proliferation
    Laura E. Schultz, Jeffrey A. Haltom, Maira P. Almeida, Wesley A. Wierson, Staci L. Solin, Trevor J. Weiss, Jordan A. Helmer, Elizabeth J. Sandquist, Heather R. Shive, Maura McGrail
    Disease Models & Mechanisms 2018 11: dmm034124 doi: 10.1242/dmm.034124 Published 15 June 2018

    Summary: This study shows that chromatin remodelers that are overexpressed in a zebrafish model of RB1 mutant brain cancer are required for neural progenitor proliferation and survival, providing insight into potential mechanisms that drive tumor growth.

  • Open Access
    Increased FGF8 signaling promotes chondrogenic rather than osteogenic development in the embryonic skull
    Linnea Schmidt, Aftab Taiyab, Vida Senkus Melvin, Kenneth L. Jones, Trevor Williams
    Disease Models & Mechanisms 2018 11: dmm031526 doi: 10.1242/dmm.031526 Published 15 June 2018

    Summary: Cranial ossification responds to Fgf8 overexpression in a dose-dependent manner with moderate levels leading to craniosynostosis and higher levels shifting cranial vault ossification to abnormal cartilage formation.

  • Open Access
    Etiology and treatment of adrenoleukodystrophy: new insights from Drosophila
    Hannah B. Gordon, Lourdes Valdez, Anthea Letsou
    Disease Models & Mechanisms 2018 11: dmm031286 doi: 10.1242/dmm.031286 Published 15 June 2018

    Summary: Using a Drosophila model of adrenoleukodystrophy, the authors provide evidence against the current view that an accumulation of lipid metabolic pathway precursors is causative of this neurometabolic disease. Rather, a lack of pathway product is the causative factor and addition of medium-chain fatty acids to the diet prevented the onset of neurodegeneration.

  • Open Access
    Impaired β-cell glucokinase as an underlying mechanism in diet-induced diabetes
    Brian Lu, Kiran Kurmi, Miguel Munoz-Gomez, Egon J. Jacobus Ambuludi, Jason M. Tonne, Kuntol Rakshit, Taro Hitosugi, Yogish C. Kudva, Aleksey V. Matveyenko, Yasuhiro Ikeda
    Disease Models & Mechanisms 2018 11: dmm033316 doi: 10.1242/dmm.033316 Published 13 June 2018

    Summary: β-cell glucokinase expression is decreased in diet-induced diabetes. β-cell-targeted overexpression of glucokinase improved the diabetic phenotype, suggesting an etiological role of glucokinase downregulation in diet-induced diabetes.

  • Open Access
    Lifespan analysis of brain development, gene expression and behavioral phenotypes in the Ts1Cje, Ts65Dn and Dp(16)1/Yey mouse models of Down syndrome
    Nadine M. Aziz, Faycal Guedj, Jeroen L. A. Pennings, Jose Luis Olmos-Serrano, Ashley Siegel, Tarik F. Haydar, Diana W. Bianchi
    Disease Models & Mechanisms 2018 11: dmm031013 doi: 10.1242/dmm.031013 Published 12 June 2018

    Editor's choice: Comparison of corticogenesis, gene expression and behavior in three mouse models of Down syndrome revealed major differences between the models as well as significant limitations in each strain for understanding neurobiological changes in the human phenotype.

  • Open Access
    Nmnat mitigates sensory dysfunction in a Drosophila model of paclitaxel-induced peripheral neuropathy
    Jennifer M. Brazill, Beverley Cruz, Yi Zhu, R. Grace Zhai
    Disease Models & Mechanisms 2018 11: dmm032938 doi: 10.1242/dmm.032938 Published 12 June 2018

    Summary: Neurotoxic side effects of chemotherapy are poorly understood. Here, the authors optimize a Drosophila model of paclitaxel-induced sensory dysfunction, which is then used to explore the neuroprotective capacity of Nmnat.

  • Open Access
    A novel rabbit model of Duchenne muscular dystrophy generated by CRISPR/Cas9
    Tingting Sui, Yeh Siang Lau, Di Liu, Tingjun Liu, Li Xu, Yandi Gao, Liangxue Lai, Zhanjun Li, Renzhi Han
    Disease Models & Mechanisms 2018 11: dmm032201 doi: 10.1242/dmm.032201 Published 4 June 2018

    Summary: The DMD KO rabbit engineered by CRISPR genome editing faithfully recapitulates the DMD pathologies, and could be a valuable tool for basic and translational studies to combat this disease.

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  • Open Access

    Altered cytoskeletal arrangement in induced pluripotent stem cells (iPSCs) and motor neurons from patients with riboflavin transporter deficiency

    Alessia Niceforo, Chiara Marioli, Fiorella Colasuonno, Stefania Petrini, Keith Massey, Marco Tartaglia, Enrico Bertini, Sandra Moreno, Claudia Compagnucci
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  • Open Access

    Genetic background modifies vulnerability to glaucoma related phenotypes in Lmx1b mutant mice

    N. G. Tolman, R. Balasubramanian, D. G. Macalinao, A. L. Kearney, K. H. MacNicoll, C. L. Montgomery, W. N. de Vries, I. J. Jackson, S. H. Cross, K. Kizhatil, K. S. Nair, S. W. M. John
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  • Open Access

    Neural crest-specific deletion of Bmp7 leads to midfacial hypoplasia, nasal airway obstruction, and disordered breathing modelling Obstructive Sleep Apnea

    Pranidhi Baddam, Vivian Biancardi, Daniela M. Roth, Farah Eaton, Claudine Thereza-Bussolaro, Rupasri Mandal, David S. Wishart, Amy Barr, Joanna MacLean, Carlos Flores-Mir, Silvia Pagliardini, Daniel Graf
    PDF
  • Open Access

    Inducible expression of human C9ORF72 36x G4C2 hexanucleotide repeats is sufficient to cause RAN translation and rapid muscular atrophy in mice

    F. W. Riemslagh, E. C. van der Toorn, R. F. M. Verhagen, A. Maas, L. W. J. Bosman, R. K. Hukema, R. Willemsen
    PDF
  • Open Access

    A muscle growth promoting treatment based on the attenuation of activin/myostatin signalling in young mice results in long-term testicular abnormalities

    Danielle Vaughan, Robert Mitchell, Oliver Kretz, David Chambers, Maciej Lalowski, Helge Amthor, Olli Ritvos, Arja Pasternack, Antonios Matsakas, Sakthivel Vaiyapuri, Tobias B. Huber, Bernd Denecke, Abir Mukherjee, Darius Widera, Ketan Patel
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