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  Open access

April, 2017; 10 (4)

EDITORIAL

  • Open Access
    Rethinking cancer: current challenges and opportunities in cancer research
    Ross Cagan, Pablo Meyer
    Disease Models & Mechanisms 2017 10: 349-352; doi: 10.1242/dmm.030007

    Summary: Pablo Meyer and Ross Cagan discuss the main themes emerging from a recent workshop ‘Rethinking Cancer’, highlighting the key challenges faced by the research community and outlining potential strategies to promote translation of basic findings.

A MODEL FOR LIFE

  • Open Access
    Waging war against pancreatic cancer: an interview with David Tuveson
    David Tuveson
    Disease Models & Mechanisms 2017 10: 353-357; doi: 10.1242/dmm.029975

    Summary: David Tuveson describes some of the key moments and breakthroughs in his remarkable career, and the path that he's followed in his quest for a cure for pancreatic cancer.

REVIEWS

  • Open Access
    In vivo models in breast cancer research: progress, challenges and future directions
    Ingunn Holen, Valerie Speirs, Bethny Morrissey, Karen Blyth
    Disease Models & Mechanisms 2017 10: 359-371; doi: 10.1242/dmm.028274

    Summary: This Review provides a summary of the many varied uses of mouse models in breast cancer research focusing on their strengths, challenges and future directions.

  • Open Access
    Identifying genes for neurobehavioural traits in rodents: progress and pitfalls
    Amelie Baud, Jonathan Flint
    Disease Models & Mechanisms 2017 10: 373-383; doi: 10.1242/dmm.027789

    Summary: This Review discusses the rodent resources that exist to map neurobehavioural traits at high resolution and identify causal variants and genes.

RESEARCH ARTICLES

  • Open Access
    MAPK signaling pathways and HDAC3 activity are disrupted during differentiation of emerin-null myogenic progenitor cells
    Carol M. Collins, Joseph A. Ellis, James M. Holaska
    Disease Models & Mechanisms 2017 10: 385-397; doi: 10.1242/dmm.028787

    Editors' choice: HDAC3, p38 MAPK and ERK signaling are altered during differentiation of myogenic progenitors lacking emerin; pharmacological activation or inhibition of these signaling proteins rescues specific stages of myogenic differentiation.

  • Open Access
    A Drosophila model of myeloproliferative neoplasm reveals a feed-forward loop in the JAK pathway mediated by p38 MAPK signalling
    Ana Terriente-Félix, Lidia Pérez, Sarah J. Bray, Angel R. Nebreda, Marco Milán
    Disease Models & Mechanisms 2017 10: 399-407; doi: 10.1242/dmm.028118

    Summary: Pro-tumourigenic crosstalk occurs between the p38 MAPK pathway and JAK signalling in a Drosophila model of myeloproliferative neoplasm.

  • Open Access
    Heterozygous Vangl2Looptail mice reveal novel roles for the planar cell polarity pathway in adult lung homeostasis and repair
    Thanushiyan Poobalasingam, Laura L. Yates, Simone A. Walker, Miguel Pereira, Nina Y. Gross, Akmol Ali, Maria Kolatsi-Joannou, Marjo-Riitta Jarvelin, Juha Pekkanen, Eugenia Papakrivopoulou, David A. Long, Mark Griffiths, Darcy Wagner, Melanie Königshoff, Matthew Hind, Cosetta Minelli, Clare M. Lloyd, Charlotte H. Dean
    Disease Models & Mechanisms 2017 10: 409-423; doi: 10.1242/dmm.028175

    Summary: Manipulating the PCP pathway may provide new approaches to treat damaged lung tissue.

  • Open Access
    Gene expression profiles among murine strains segregate with distinct differences in the progression of radiation-induced lung disease
    Isabel L. Jackson, Fitsum Baye, Chirayu P. Goswami, Barry P. Katz, Andrew Zodda, Radmila Pavlovic, Ganga Gurung, Don Winans, Zeljko Vujaskovic
    Disease Models & Mechanisms 2017 10: 425-437; doi: 10.1242/dmm.028217

    Summary: Rational mouse model selection is crucial for identifying new therapeutic targets and screening medical interventions in acute pneumonitis and/or late fibrosis following thoracic irradiation.

  • Open Access
    Overexpression of mitochondrial oxodicarboxylate carrier (ODC1) preserves oxidative phosphorylation in a yeast model of Barth syndrome
    Maxence de Taffin de Tilques, Déborah Tribouillard-Tanvier, Emmanuel Tétaud, Eric Testet, Jean-Paul di Rago, Jean-Paul Lasserre
    Disease Models & Mechanisms 2017 10: 439-450; doi: 10.1242/dmm.027540

    Summary: Transport of oxodicarboxylic acid (ODC1) across the mitochondrial inner membrane is a potential target for the treatment of BTHS patients and other diseases caused by mitochondrial dysfunction.

  • Open Access
    Towards trans-diagnostic mechanisms in psychiatry: neurobehavioral profile of rats with a loss-of-function point mutation in the dopamine transporter gene
    Valentina Vengeliene, Anton Bespalov, Martin Roßmanith, Sandra Horschitz, Stefan Berger, Ana L. Relo, Hamid R. Noori, Peggy Schneider, Thomas Enkel, Dusan Bartsch, Miriam Schneider, Berthold Behl, Anita C. Hansson, Patrick Schloss, Rainer Spanagel
    Disease Models & Mechanisms 2017 10: 451-461; doi: 10.1242/dmm.027623

    Summary: The first systematic RDoc study of a disease mechanism proposes dopamine transporter DAT mutant rats as a model for drug development, targeting a hyperdopaminergic state.

  • Open Access
    New insights into the regulatory function of CYFIP1 in the context of WAVE- and FMRP-containing complexes
    Sabiha Abekhoukh, H. Bahar Sahin, Mauro Grossi, Samantha Zongaro, Thomas Maurin, Irene Madrigal, Daniele Kazue-Sugioka, Annick Raas-Rothschild, Mohamed Doulazmi, Pilar Carrera, Andrea Stachon, Steven Scherer, Maria Rita Drula Do Nascimento, Alain Trembleau, Ignacio Arroyo, Peter Szatmari, Isabel M. Smith, Montserrat Milà, Adam C. Smith, Angela Giangrande, Isabelle Caillé, Barbara Bardoni
    Disease Models & Mechanisms 2017 10: 463-474; doi: 10.1242/dmm.025809

    Summary: CYFIP1 and FMRP interact antagonistically during neuromuscular junction growth in the fly and during new neuron differentiation in the olfactory bulb of the adult mouse.

  • Open Access
    Genetic dissection of anterior segment dysgenesis caused by a Col4a1 mutation in mouse
    Mao Mao, Márton Kiss, Yvonne Ou, Douglas B. Gould
    Disease Models & Mechanisms 2017 10: 475-485; doi: 10.1242/dmm.027888

    Summary: Key pathogenic events in anterior segment dysgenesis, a congenital ocular disease with complex etiology, are recapitulated in a mouse model of Col4a1-related ASD.

RESOURCE ARTICLE

  • Open Access
    Immortalized human myotonic dystrophy muscle cell lines to assess therapeutic compounds
    Ludovic Arandel, Micaela Polay Espinoza, Magdalena Matloka, Audrey Bazinet, Damily De Dea Diniz, Naïra Naouar, Frédérique Rau, Arnaud Jollet, Frédérique Edom-Vovard, Kamel Mamchaoui, Mark Tarnopolsky, Jack Puymirat, Christophe Battail, Anne Boland, Jean-Francois Deleuze, Vincent Mouly, Arnaud F. Klein, Denis Furling
    Disease Models & Mechanisms 2017 10: 487-497; doi: 10.1242/dmm.027367

    Summary: Myotonic dystrophy muscle cell models displaying characteristic disease-associated molecular features can be used to investigate molecular pathophysiological mechanisms and evaluate therapeutic approaches.

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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
    PDF
  • 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
    PDF
  • 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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Professor Elizabeth Patton appointed as DMM’s next Editor-in-Chief

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Identification of MYOM2 as a candidate gene in hypertrophic cardiomyopathy and Tetralogy of Fallot, and its functional evaluation in the Drosophila heart

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C. elegans as a disease model

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Call for papers – The RAS Pathway: Diseases, Therapeutics and Beyond

Our upcoming special issue is now welcoming submissions until 1 April 2021. Guest-edited by Donita Brady (Perelman School of Medicine at the University of Pennsylvania, USA) and Arvin Dar (Icahn School of Medicine at Mount Sinai, USA), the issue will focus on the targeting the RAS pathway. Find out more about the issue and how to submit your manuscript.


Interview – Kim Landry-Truchon and Nicolas Houde

In an interview, first authors Kim Landry-Truchon and Nicolas Houde discuss their mouse model of the early stages of pleuropulmonary blastoma, reflecting on the implications of their work and the future of their field.

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