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Subject collection: Neurodegenerative Disorders

  • REVIEW
    Modelling amyotrophic lateral sclerosis: progress and possibilities
    Philip Van Damme, Wim Robberecht, Ludo Van Den Bosch
    Disease Models & Mechanisms 2017 10: 537-549; doi: 10.1242/dmm.029058

    Summary: In this Review, Ludo Van Den Bosch and colleagues discuss the different model systems for studying ALS and how they have contributed to our current understanding of the etiology and pathology of this neurodegenerative disease.

  • RESEARCH ARTICLES
    Upregulation of CB2 receptors in reactive astrocytes in canine degenerative myelopathy, a disease model of amyotrophic lateral sclerosis
    María Fernández-Trapero, Francisco Espejo-Porras, Carmen Rodríguez-Cueto, Joan R. Coates, Carmen Pérez-Díaz, Eva de Lago, Javier Fernández-Ruiz
    Disease Models & Mechanisms 2017 10: 551-558; doi: 10.1242/dmm.028373

    Editors' choice: CB2 receptors are upregulated in activated astrocytes recruited at the damaged spinal cord in dogs with degenerative myelopathy, a canine model of amyotrophic lateral sclerosis.

  • RESEARCH ARTICLES
    Loss of Ranbp2 in motoneurons causes disruption of nucleocytoplasmic and chemokine signaling, proteostasis of hnRNPH3 and Mmp28, and development of amyotrophic lateral sclerosis-like syndromes
    Kyoung-in Cho, Dosuk Yoon, Sunny Qiu, Zachary Danziger, Warren M. Grill, William C. Wetsel, Paulo A. Ferreira
    Disease Models & Mechanisms 2017 10: 559-579; doi: 10.1242/dmm.027730

    Summary: Loss of Ranbp2 in spinal motoneurons drives ALS syndromes in mice and Ranbp2 functions in nucleocytoplasmic trafficking, proteostasis and chemokine signaling uncover novel therapeutic targets and mechanisms for motoneuron disease.

  • RESEARCH ARTICLES
    The tyrosine kinase receptor Tyro3 enhances lifespan and neuropeptide Y (Npy) neuron survival in the mouse anorexia (anx) mutation
    Dennis Y. Kim, Joanna Yu, Ryan K. Mui, Rieko Niibori, Hamza Bin Taufique, Rukhsana Aslam, John W. Semple, Sabine P. Cordes
    Disease Models & Mechanisms 2017 10: 581-595; doi: 10.1242/dmm.027433

    Summary: The receptor tyrosine kinase Tyro3 improves weight and survival in the anorexia mouse model, and is one of the few factors known to sustain appetite regulatory circuitry.

  • RESEARCH ARTICLES
    Schwann cells are activated by ATP released from neurons in an in vitro cellular model of Miller Fisher syndrome
    Umberto Rodella, Samuele Negro, Michele Scorzeto, Elisanna Bergamin, Kees Jalink, Cesare Montecucco, Nobuhiro Yuki, Michela Rigoni
    Disease Models & Mechanisms 2017 10: 597-603; doi: 10.1242/dmm.027870

    Summary: ATP released by degenerating neurons participates in neuron-Schwann cell communication in an in vitro model of Miller Fisher syndrome and activates Schwann cell pro-regenerative properties.

  • RESEARCH ARTICLES
    The familial dysautonomia disease gene IKBKAP is required in the developing and adult mouse central nervous system
    Marta Chaverra, Lynn George, Marc Mergy, Hannah Waller, Katharine Kujawa, Connor Murnion, Ezekiel Sharples, Julian Thorne, Nathaniel Podgajny, Andrea Grindeland, Yumi Ueki, Steven Eiger, Cassie Cusick, A. Michael Babcock, George A. Carlson, Frances Lefcort
    Disease Models & Mechanisms 2017 10: 605-618; doi: 10.1242/dmm.028258

    Summary: Ikbkap is essential for normal CNS development, neuronal survival and behavior, adding to our understanding of the role of the Elongator complex in the mammalian CNS.

  • RESEARCH ARTICLES
    Blood RNA biomarkers in prodromal PARK4 and rapid eye movement sleep behavior disorder show role of complexin 1 loss for risk of Parkinson's disease
    Suna Lahut, Suzana Gispert, Özgür Ömür, Candan Depboylu, Kay Seidel, Jorge Antolio Domínguez-Bautista, Nadine Brehm, Hülya Tireli, Karl Hackmann, Caroline Pirkevi, Barbara Leube, Vincent Ries, Kerstin Reim, Nils Brose, Wilfred F. den Dunnen, Madrid Johnson, Zsuzsanna Wolf, Marc Schindewolf, Wiebke Schrempf, Kathrin Reetz, Peter Young, David Vadasz, Achilleas S. Frangakis, Evelin Schröck, Helmuth Steinmetz, Marina Jendrach, Udo Rüb, Ayşe Nazlı Başak, Wolfgang Oertel, Georg Auburger
    Disease Models & Mechanisms 2017 10: 619-631; doi: 10.1242/dmm.028035

    Summary: Complexin 1 is a prodromal biomarker and risk factor for REM sleep behavior disorder and PARK4-associated Parkinson's disease.

  • RESEARCH ARTICLES
    Genetic mutations linked to Parkinson's disease differentially control nucleolar activity in pre-symptomatic mouse models
    Valentin Evsyukov, Andrii Domanskyi, Holger Bierhoff, Suzana Gispert, Rasem Mustafa, Falk Schlaudraff, Birgit Liss, Rosanna Parlato
    Disease Models & Mechanisms 2017 10: 633-643; doi: 10.1242/dmm.028092

    Summary: Genetic mutations linked to Parkinson's disease lead to stage-specific deregulation of the nucleolus, a major integrator of the cellular stress response.

  • RESEARCH ARTICLES
    A differential autophagy-dependent response to DNA double-strand breaks in bone marrow mesenchymal stem cells from sporadic ALS patients
    Shane Wald-Altman, Edward Pichinuk, Or Kakhlon, Miguel Weil
    Disease Models & Mechanisms 2017 10: 645-654; doi: 10.1242/dmm.027938

    Summary: A novel endogenous disease mechanism in cells from ALS patients after NCS-induced DNA damage.

  • RESEARCH ARTICLES
    Cellular levels of Grb2 and cytoskeleton stability are correlated in a neurodegenerative scenario
    Piyali Majumder, Kasturi Roy, Brijesh Kumar Singh, Nihar Ranjan Jana, Debashis Mukhopadhyay
    Disease Models & Mechanisms 2017 10: 655-669; doi: 10.1242/dmm.027748

    Summary: Grb2 has a unique role in protecting the cytoskeletal architecture in AD-like conditions, offering a potential new strategy for controlling neurodegeneration.

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