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  Open access

January, 2014; 7 (1)

IN THIS ISSUE

  • Open Access
    Promoting skeletal muscle regeneration in mouse and fly models of DMD
    Disease Models & Mechanisms 2014 7: 1
  • Open Access
    Drug discovery for neutrophil-mediated inflammation
    Disease Models & Mechanisms 2014 7: 1
  • Open Access
    A nutrient-sensing pathway activated in APC-driven colorectal cancer
    Disease Models & Mechanisms 2014 7: 1
  • Open Access
    A combinatorial therapy for treating obesity
    Disease Models & Mechanisms 2014 7: 1
  • Open Access
    A protective role for the UPR in galactosaemia
    Disease Models & Mechanisms 2014 7: 1
  • Open Access
    Phosphatidylinositol signalling defects implicated in IBD
    Disease Models & Mechanisms 2014 7: 1

EDITORIAL

  • Open Access
    The threads that tie protein-folding diseases
    Jeffrey L. Brodsky
    Disease Models & Mechanisms 2014 7: 3-4; doi: 10.1242/dmm.014985

A MODEL FOR LIFE

  • Open Access
    Creating a path from the heat shock response to therapeutics of protein-folding diseases: an interview with Rick Morimoto
    Disease Models & Mechanisms 2014 7: 5-8; doi: 10.1242/dmm.014753

AT A GLANCE

  • Open Access
    Mechanisms of protein-folding diseases at a glance
    Julie S. Valastyan, Susan Lindquist
    Disease Models & Mechanisms 2014 7: 9-14; doi: 10.1242/dmm.013474

REVIEWS

  • Open Access
    Aneuploidy: implications for protein homeostasis and disease
    Ana B. Oromendia, Angelika Amon
    Disease Models & Mechanisms 2014 7: 15-20; doi: 10.1242/dmm.013391
  • Open Access
    Selective vulnerability to neurodegenerative disease: the curious case of Prion Protein
    Walker S. Jackson
    Disease Models & Mechanisms 2014 7: 21-29; doi: 10.1242/dmm.012146
  • Open Access
    Caenorhabditis elegans as a model system for studying non-cell-autonomous mechanisms in protein-misfolding diseases
    Carmen I. Nussbaum-Krammer, Richard I. Morimoto
    Disease Models & Mechanisms 2014 7: 31-39; doi: 10.1242/dmm.013011

RESEARCH ARTICLES

  • Open Access
    Molecular mechanism of sphingosine-1-phosphate action in Duchenne muscular dystrophy
    Diem-Hang Nguyen-Tran, Nitai C. Hait, Henrik Sperber, Junlin Qi, Karin Fischer, Nick Ieronimakis, Mario Pantoja, Aislinn Hays, Jeremy Allegood, Morayma Reyes, Sarah Spiegel, Hannele Ruohola-Baker
    Disease Models & Mechanisms 2014 7: 41-54; doi: 10.1242/dmm.013631
  • Open Access
    The unfolded protein response has a protective role in yeast models of classic galactosemia
    Evandro A. De-Souza, Felipe S. A. Pimentel, Caio M. Machado, Larissa S. Martins, Wagner S. da-Silva, Mónica Montero-Lomelí, Claudio A. Masuda
    Disease Models & Mechanisms 2014 7: 55-61; doi: 10.1242/dmm.012641
  • Open Access
    Oncogenic mutations in adenomatous polyposis coli (Apc) activate mechanistic target of rapamycin complex 1 (mTORC1) in mice and zebrafish
    Alexander J. Valvezan, Jian Huang, Christopher J. Lengner, Michael Pack, Peter S. Klein
    Disease Models & Mechanisms 2014 7: 63-71; doi: 10.1242/dmm.012625
  • Open Access
    A new zebrafish model produced by TILLING of SOD1-related amyotrophic lateral sclerosis replicates key features of the disease and represents a tool for in vivo therapeutic screening
    Marc M. J. Da Costa, Claire E. Allen, Adrian Higginbottom, Tennore Ramesh, Pamela J. Shaw, Christopher J. McDermott
    Disease Models & Mechanisms 2014 7: 73-81; doi: 10.1242/dmm.012013
  • Open Access
    TGF-β3 modulates the inflammatory environment and reduces scar formation following vocal fold mucosal injury in rats
    Zhen Chang, Yo Kishimoto, Ayesha Hasan, Nathan V. Welham
    Disease Models & Mechanisms 2014 7: 83-91; doi: 10.1242/dmm.013326
  • Open Access
    Dysregulated phosphatidylinositol signaling promotes endoplasmic-reticulum-stress-mediated intestinal mucosal injury and inflammation in zebrafish
    Prakash C. Thakur, Jon M. Davison, Carsten Stuckenholz, Lili Lu, Nathan Bahary
    Disease Models & Mechanisms 2014 7: 93-106; doi: 10.1242/dmm.012864
  • Open Access
    Valproic acid silencing of ascl1b/Ascl1 results in the failure of serotonergic differentiation in a zebrafish model of fetal valproate syndrome
    John Jacob, Vanessa Ribes, Steven Moore, Sean C. Constable, Noriaki Sasai, Sebastian S. Gerety, Darren J. Martin, Chris P. Sergeant, David G. Wilkinson, James Briscoe
    Disease Models & Mechanisms 2014 7: 107-117; doi: 10.1242/dmm.013219
  • Open Access
    A missense mutation accelerating the gating of the lysosomal Cl−/H+-exchanger ClC-7/Ostm1 causes osteopetrosis with gingival hamartomas in cattle
    Arnaud Sartelet, Tobias Stauber, Wouter Coppieters, Carmen F. Ludwig, Corinne Fasquelle, Tom Druet, Zhiyan Zhang, Naima Ahariz, Nadine Cambisano, Thomas J. Jentsch, Carole Charlier
    Disease Models & Mechanisms 2014 7: 119-128; doi: 10.1242/dmm.012500
  • Open Access
    Oleoylethanolamide enhances β-adrenergic-mediated thermogenesis and white-to-brown adipocyte phenotype in epididymal white adipose tissue in rat
    Juan Suárez, Patricia Rivera, Sergio Arrabal, Ana Crespillo, Antonia Serrano, Elena Baixeras, Francisco J. Pavón, Manuel Cifuentes, Rubén Nogueiras, Joan Ballesteros, Carlos Dieguez, Fernando Rodríguez de Fonseca
    Disease Models & Mechanisms 2014 7: 129-141; doi: 10.1242/dmm.013110
  • Open Access
    Transcriptional changes and developmental abnormalities in a zebrafish model of myotonic dystrophy type 1
    Peter K. Todd, Feras Y. Ackall, Junguk Hur, Kush Sharma, Henry L. Paulson, James J. Dowling
    Disease Models & Mechanisms 2014 7: 143-155; doi: 10.1242/dmm.012427

RESEARCH REPORTS

  • Open Access
    The myopathy-causing mutation DNM2-S619L leads to defective tubulation in vitro and in developing zebrafish
    Elizabeth M. Gibbs, Ann E. Davidson, William R. Telfer, Eva L. Feldman, James J. Dowling
    Disease Models & Mechanisms 2014 7: 157-161; doi: 10.1242/dmm.012286
  • Open Access
    Inhibitors of neutrophil recruitment identified using transgenic zebrafish to screen a natural product library
    Xingang Wang, Anne L. Robertson, Jingyu Li, Ruth Jinfen Chai, Wang Haishan, Pranvera Sadiku, Nikolay V. Ogryzko, Martin Everett, Kanagasundaram Yoganathan, Hongbo Robert Luo, Stephen A. Renshaw, Philip W. Ingham
    Disease Models & Mechanisms 2014 7: 163-169; doi: 10.1242/dmm.012047
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Disease Models & Mechanisms: 7 (1)
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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
    PDF
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Interview – Kim Landry-Truchon and Nicolas Houde

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