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  Open access

March, 2018; 11 (3)

REVIEW

  • Open Access
    Metastasis in context: modeling the tumor microenvironment with cancer-on-a-chip approaches
    Jelle J. F. Sleeboom, Hossein Eslami Amirabadi, Poornima Nair, Cecilia M. Sahlgren, Jaap M. J. den Toonder
    Disease Models & Mechanisms 2018 11: dmm033100 doi: 10.1242/dmm.033100 Published 16 March 2018

    Summary: This Review evaluates the recent contributions of cancer-on-a-chip models to our understanding of the tumor microenvironment and its role in the onset of metastasis. The authors also provide an outlook for future applications of this emerging technology.

RESEARCH ARTICLES

  • Open Access
    Neutrophil-specific knockout demonstrates a role for mitochondria in regulating neutrophil motility in zebrafish
    Wenqing Zhou, Lingyan Cao, Jacob Jeffries, Xiaoguang Zhu, Christopher J. Staiger, Qing Deng
    Disease Models & Mechanisms 2018 11: dmm033027 doi: 10.1242/dmm.033027 Published 28 March 2018

    Summary: Using CRISPR/Cas9-mediated neutrophil-specific knockout, a requirement for mitochondrial DNA polymerase, electron transportation chain and superoxide dismutases in neutrophil migration is demonstrated in zebrafish, providing genetic evidence of the immunodeficiency seen in mitochondrial disease patients.

  • Open Access
    Feeding difficulties, a key feature of the Drosophila NDUFS4 mitochondrial disease model
    Sarah Foriel, Julien Beyrath, Ilse Eidhof, Richard J. Rodenburg, Annette Schenck, Jan A. M. Smeitink
    Disease Models & Mechanisms 2018 11: dmm032482 doi: 10.1242/dmm.032482 Published 27 March 2018

    Summary: The characterization of a novel Drosophila model of NDUFS4 mitochondrial disease revealed a pronounced defect in feeding abilities and recapitulated additional human disease features.

  • Open Access
    Lymphoblastoid cell lines as a model to understand amyotrophic lateral sclerosis disease mechanisms
    Orietta Pansarasa, Matteo Bordoni, Lorenzo Drufuca, Luca Diamanti, Daisy Sproviero, Rosa Trotti, Stefano Bernuzzi, Sabrina La Salvia, Stella Gagliardi, Mauro Ceroni, Cristina Cereda
    Disease Models & Mechanisms 2018 11: dmm031625 doi: 10.1242/dmm.031625 Published 26 March 2018

    Summary: The identification of a patient-specific and non-invasive model is crucial in ALS research. Here, the authors present a reliable characterization of lymphoblastoid cell lines from both sporadic and mutated ALS patients.

  • Open Access
    The role of the microRNA-146a/complement factor H/interleukin-1β-mediated inflammatory loop circuit in the perpetuate inflammation of chronic temporal lobe epilepsy
    Tao-Ran Li, Yan-Jie Jia, Chao Ma, Wen-Ying Qiu, Qun Wang, Xiao-Qiu Shao, Rui-Juan Lv
    Disease Models & Mechanisms 2018 11: dmm031708 doi: 10.1242/dmm.031708 Published 23 March 2018

    Summary: The microRNA-146a–complement factor H–interleukin-1β loop circuit might initiate a cascade of inflammation, leading to the perpetuate inflammation in temporal lobe epilepsy.

  • Open Access
    Vangl2 disruption alters the biomechanics of late spinal neurulation leading to spina bifida in mouse embryos
    Gabriel L. Galea, Oleksandr Nychyk, Matteo A. Mole, Dale Moulding, Dawn Savery, Evanthia Nikolopoulou, Deborah J. Henderson, Nicholas D. E. Greene, Andrew J. Copp
    Disease Models & Mechanisms 2018 11: dmm032219 doi: 10.1242/dmm.032219 Published 21 March 2018

    Summary: Disruption of Vangl2-dependent planar-polarised processes in the posterior neuropore (PNP) neuroepithelium and surface ectoderm preclude zippering point biomechanical accommodation associated with Closure 5 formation at the completion of PNP closure.

  • Open Access
    Identification of protective postexposure mycobacterial vaccine antigens using an immunosuppression-based reactivation model in the zebrafish
    Henna Myllymäki, Mirja Niskanen, Hanna Luukinen, Mataleena Parikka, Mika Rämet
    Disease Models & Mechanisms 2018 11: dmm033175 doi: 10.1242/dmm.033175 Published 13 March 2018

    Summary: We characterize the reactivation of a latent mycobacterial infection and identify DNA-based vaccine candidates against the reactivation of tuberculosis using the adult zebrafish Mycobacterium marinum infection model.

  • Open Access
    Mek1Y130C mice recapitulate aspects of human cardio-facio-cutaneous syndrome
    Rifdat Aoidi, Nicolas Houde, Kim Landry-Truchon, Michael Holter, Kevin Jacquet, Louis Charron, Suguna Rani Krishnaswami, Benjamin D. Yu, Katherine A. Rauen, Nicolas Bisson, Jason Newbern, Jean Charron
    Disease Models & Mechanisms 2018 11: dmm031278 doi: 10.1242/dmm.031278 Published 13 March 2018

    Summary: A mouse model for cardio-facio-cutaneous syndrome caused by MEK1 Y130C mutant protein reveals the role of hyperactivation of the RAS/MAPK pathway in the development of the syndrome.

  • Open Access
    Altered expression of the Cdk5 activator-like protein, Cdk5α, causes neurodegeneration, in part by accelerating the rate of aging
    Joshua Spurrier, Arvind Kumar Shukla, Kristina McLinden, Kory Johnson, Edward Giniger
    Disease Models & Mechanisms 2018 11: dmm031161 doi: 10.1242/dmm.031161 Published 8 March 2018

    Editor's choice: By developing a comprehensive and quantitative metric for physiological age of Drosophila, we show that a neurodegeneration mutant produces its effects in part by accelerating the absolute rate of aging.

  • Open Access
    Vitamin D deficiency in the ApcPirc/+ rat does not exacerbate colonic tumorigenesis, while low dietary calcium might be protective
    Amy A. Irving, Elizabeth G. Duchow, Lori A. Plum, Hector F. DeLuca
    Disease Models & Mechanisms 2018 11: dmm032300 doi: 10.1242/dmm.032300 Published 7 March 2018

    Summary: Using rat models of familial and inflammation-induced colon cancer, this study demonstrates that vitamin D deficiency does not increase risk of developing disease, and that low dietary calcium might be protective.

  • Open Access
    Identification of early indicators of altered metabolism in normal development using a rodent model system
    Ashok Daniel Prabakaran, Jimsheena Valiyakath Karakkat, Ranjit Vijayan, Jisha Chalissery, Marwa F. Ibrahim, Suneesh Kaimala, Ernest A. Adeghate, Ahmed Hassan Al-Marzouqi, Suraiya Anjum Ansari, Eric Mensah-Brown, Bright Starling Emerald
    Disease Models & Mechanisms 2018 11: dmm031815 doi: 10.1242/dmm.031815 Published 1 March 2018

    Summary: This study suggests an important link between the early embryonic environment and later adult physiology and pathology. At least one process by which this might be coordinated is through the regulatory mechanisms mediated by Dnmt1.

RESOURCE ARTICLE

  • Open Access
    Disease Ontology: improving and unifying disease annotations across species
    Susan M. Bello, Mary Shimoyama, Elvira Mitraka, Stanley J. F. Laulederkind, Cynthia L. Smith, Janan T. Eppig, Lynn M. Schriml
    Disease Models & Mechanisms 2018 11: dmm032839 doi: 10.1242/dmm.032839 Published 12 March 2018

    Summary: Analyzing diverse disease data requires a comprehensive, robust disease ontology to integrate annotations and retrieve accurate, interpretable results. MGD, RGD and DO are working in collaboration to achieve this goal.

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  • Open Access

    Modelling epilepsy in the mouse: challenges and solutions

    Grant F. Marshall, Alfredo Gonzalez-Sulser, Catherine M. Abbott
    PDF
  • Open Access

    EZH2 is required for parathyroid and thymic development through differentiation of the third pharyngeal pouch endoderm

    Cinzia Caprio, Gabriella Lania, Marchesa Bilio, Rosa Ferrentino, Li Chen, Antonio Baldini
    PDF
  • Open Access

    High-dose vitamin B1 therapy prevents the development of experimental fatty liver driven by overnutrition

    Mugagga Kalyesubula, Ramgopal Mopuri, Jimmy Asiku, Alexander Rosov, Sara Yosefi, Nir Edery, Samuel Bocobza, Uzi Moallem, Hay Dvir
    PDF
  • Open Access

    Transformed notochordal cells trigger chronic wounds destabilizing the vertebral column and bone homeostasis

    Paco López-Cuevas, Luke Deane, Yushi Yang, Chrissy L Hammond, Erika Kague
    PDF
  • Open Access

    Multiscale molecular profiling of pathological bone resolves sexually dimorphic control of extracellular matrix composition

    Aikta Sharma, Alice Goring, Peter B. Johnson, Roger J. H. Emery, Eric Hesse, Alan Boyde, Bjorn R. Olsen, Andrew A. Pitsillides, Richard O. C. Oreffo, Sumeet Mahajan, Claire E. Clarkin
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Monica Justice bids farewell to DMM

In her farewell Editorial, outgoing Editor-in-Chief Monica Justice reminds us of the past half-decade of growth and of DMM's commitment to support the disease modelling community, concluding, “The knowledge and experience I gained during my time as Senior Editor and EiC at DMM is invaluable: working within a not-for-profit community publishing environment is a joy.”


3D imaging of beta cell mass in diabetic mouse models

In their inducible mouse model of diabetes, Roostalu et al. demonstrate how quantitative light-sheet imaging can capture changes in individual islets to help pharmacological research in diabetes.

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Modelling Joubert syndrome patient-derived mutations in C. elegans

In this issue’s Editor’s choice, Karen Lange and colleagues used C. elegans to model and characterise two patient-derived mutations that cause the ciliopathy Joubert syndrome.


Interview – Karen Lange

First author of our current Editor’s choice, Karen Lange takes us behind the scenes of the paper, and shares her thoughts on how the lack of both time and job security will impact her research.

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