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Subject collection: Neurodegenerative Disorders

  • RESOURCE ARTICLE
    Bar-coding neurodegeneration: identifying subcellular effects of human neurodegenerative disease proteins using Drosophila leg neurons
    Josefin Fernius, Annika Starkenberg, Stefan Thor
    Disease Models & Mechanisms 2017 10: 1027-1038; doi: 10.1242/dmm.029637

    Summary: Toxic effects of neurodegenerative disease proteins on neuronal function and morphology can be addressed using an array of transgenic fluorescent reporters in the adult Drosophila leg.

  • REVIEW
    Therapeutic strategies for spinal muscular atrophy: SMN and beyond
    Melissa Bowerman, Catherina G. Becker, Rafael J. Yáñez-Muñoz, Ke Ning, Matthew J. A. Wood, Thomas H. Gillingwater, Kevin Talbot, The UK SMA Research Consortium
    Disease Models & Mechanisms 2017 10: 943-954; doi: 10.1242/dmm.030148

    Summary: Translational research for spinal muscular atrophy (SMA) should address the development of non-CNS and survival motor neuron (SMN)-independent therapeutic approaches to complement and enhance the benefits of CNS-directed and SMN-dependent therapies.

  • RESEARCH ARTICLE
    Viral delivery of C9orf72 hexanucleotide repeat expansions in mice leads to repeat-length-dependent neuropathology and behavioural deficits
    Saul Herranz-Martin, Jayanth Chandran, Katherine Lewis, Padraig Mulcahy, Adrian Higginbottom, Callum Walker, Isabel Martinez-Pena y Valenzuela, Ross A. Jones, Ian Coldicott, Tommaso Iannitti, Mohammed Akaaboune, Sherif F. El-Khamisy, Thomas H. Gillingwater, Pamela J. Shaw, Mimoun Azzouz
    Disease Models & Mechanisms 2017 10: 859-868; doi: 10.1242/dmm.029892

    Summary: C9orf72-linked motor neuron disease models with viral-mediated expression of GGGGCC repeat expansion in mice show neuropathology and behavioural deficits.

  • REVIEW
    Induced pluripotent stem cell models of lysosomal storage disorders
    Daniel K. Borger, Benjamin McMahon, Tamanna Roshan Lal, Jenny Serra-Vinardell, Elma Aflaki, Ellen Sidransky
    Disease Models & Mechanisms 2017 10: 691-704; doi: 10.1242/dmm.029009

    Summary: This Review discusses how induced pluripotent stem cells (iPSCs) provide new opportunities to explore the biology and pathophysiology of lysosomal storage diseases, and how iPSCs have illuminated the role of lysosomes in more common disorders.

  • RESEARCH ARTICLE
    Dietary reversal of neuropathy in a murine model of prediabetes and metabolic syndrome
    Lucy M. Hinder, Phillipe D. O'Brien, John M. Hayes, Carey Backus, Andrew P. Solway, Catrina Sims-Robinson, Eva L. Feldman
    Disease Models & Mechanisms 2017 10: 717-725; doi: 10.1242/dmm.028530

    Summary: A mouse model of metabolic syndrome can be used to investigate peripheral nerve damage, enabling the development of mechanism-based therapies for neuropathy.

  • RESEARCH ARTICLE
    Characterization of Drosophila Saposin-related mutants as a model for lysosomal sphingolipid storage diseases
    Julia Sellin, Heike Schulze, Marie Paradis, Dominic Gosejacob, Cyrus Papan, Andrej Shevchenko, Olympia Ekaterina Psathaki, Achim Paululat, Melanie Thielisch, Konrad Sandhoff, Michael Hoch
    Disease Models & Mechanisms 2017 10: 737-750; doi: 10.1242/dmm.027953

    Summary: Detailed analysis of lipid accumulation, lysosomal dysfunction and neurodegeneration in a Drosophila sphingolipidosis model, suitable for studying the resulting pathological signaling events.

  • EDITORIAL
    Neurodegenerative disease: models, mechanisms, and a new hope
    Aaron D. Gitler, Paraminder Dhillon, James Shorter
    Disease Models & Mechanisms 2017 10: 499-502; doi: 10.1242/dmm.030205

    Summary: This Editorial introduces a new Special Collection, ‘Neurodegeneration: from models to mechanisms to therapies’, providing a summary of the research and review articles published in this launch issue, as well as highlighting some of DMM's most-read neurodegeneration-related articles from recent issues.

  • A MODEL FOR LIFE
    Solving the puzzle of neurological diseases: an interview with Huda Zoghbi
    Huda Y. Zoghbi
    Disease Models & Mechanisms 2017 10: 503-507; doi: 10.1242/dmm.029751

    Summary: Huda Zoghbi discusses her remarkable journey from the clinic to the bench, highlighting the experiences and collaborations that inspired her interest in neurological diseases such as spinocerebellar ataxia type 1 (SCA1) and Rett syndrome.

  • AT A GLANCE
    RNA metabolism in neurodegenerative disease
    Elaine Y. Liu, Christopher P. Cali, Edward B. Lee
    Disease Models & Mechanisms 2017 10: 509-518; doi: 10.1242/dmm.028613

    Summary: In this At a Glance review, Edward Lee and co-authors provide an overview of RNA metabolism defects, including mislocalization of RNA-binding proteins and microRNA biogenesis alterations, that contribute to neurodegenerative disease pathology.

  • REVIEW
    The role of Ca2+ signaling in Parkinson's disease
    Sofia V. Zaichick, Kaitlyn M. McGrath, Gabriela Caraveo
    Disease Models & Mechanisms 2017 10: 519-535; doi: 10.1242/dmm.028738

    Summary: This Review article discusses the important roles that calcium plays in the pathogenesis of Parkinson's disease.

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