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Neurodegeneration

A growing aging population means that age-dependent neurodegenerative disorders are affecting increasing numbers of people. Model systems have proven to be excellent tools for studying neurodegenerative processes, facilitating drug discovery in this area. DMM features reviews, research, resources, interviews and other articles, including specially commissioned content from our 2017 special issue Neurodegeneration: from Models to Mechanisms to Therapies, which was guest edited by Aaron Gitler and James Shorter.

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RESEARCH ARTICLE

OPA1 deficiency impairs oxidative metabolism in cycling cells, underlining a translational approach for degenerative diseases

Aurélie M. C. Millet; Corentin Coustham; Camille Champigny; Marlène Botella; Christine Demeilliers; Anne Devin; Anne Galinier; Pascale Belenguer; Joel Bordeneuve-Guibé; Noélie Davezac

20 September 2023

10.1242/dmm.050266

RESEARCH ARTICLE

Blood levels of neurofilament light are associated with disease progression in a mouse model of spinocerebellar ataxia type 3

David Mengel; Isabel G. Wellik; Kristen H. Schuster; Sabrina I. Jarrah; Madeleine Wacker; Naila S. Ashraf; Gülin Öz; Matthis Synofzik; Maria do Carmo Costa; Hayley S. McLoughlin

04 September 2023

10.1242/dmm.050144

RESEARCH ARTICLE

A novel porcine model of CLN3 Batten disease recapitulates clinical phenotypes

Vicki J. Swier; Katherine A. White; Tyler B. Johnson; Xiaojun Wang; Jimin Han; David A. Pearce; Ruchira Singh; Arlene V. Drack; Wanda Pfeifer; Christopher S. Rogers; Jon J. Brudvig; Jill M. Weimer

07 August 2023

10.1242/dmm.050038

RESEARCH ARTICLE

Attenuated cerebellar phenotypes in Inpp4a truncation mutants with preserved phosphatase activity

Dang Minh Tran; Nozomu Yoshioka; Norihisa Bizen; Yukiko Mori-Ochiai; Masato Yano; Shogo Yanai; Junya Hasegawa; Satoshi Miyashita; Mikio Hoshino; Junko Sasaki; Takehiko Sasaki; Hirohide Takebayashi

28 July 2023

10.1242/dmm.050169

RESEARCH ARTICLE

Unexpected phenotypic and molecular changes of combined glucocerebrosidase and acid sphingomyelinase deficiency

Marcus Keatinge; Matthew E. Gegg; Lisa Watson; Heather Mortiboys; Nan Li; Mark Dunning; Deepak Ailani; Hai Bui; Astrid van Rens; Dirk J. Lefeber; Anthony H. V. Schapira; Ryan B. MacDonald; Oliver Bandmann

10 May 2023

10.1242/dmm.049954

RESEARCH ARTICLE

Impaired episodic-like memory in a mouse model of Alzheimer's disease is associated with hyperactivity in prefrontal–hippocampal regions

Sijie Tan; Wen Han Tong; Ajai Vyas

10 March 2023

10.1242/dmm.049945

RESEARCH ARTICLE

Increased cysteine metabolism in PINK1 models of Parkinson's disease

Marco Travaglio; Filippos Michopoulos; Yizhou Yu; Rebeka Popovic; Edmund Foster; Muireann Coen; L. Miguel Martins

25 January 2023

10.1242/dmm.049727

RESEARCH ARTICLE

String/Cdc25 phosphatase is a suppressor of Tau-associated neurodegeneration

Andreia C. Oliveira; Madalena Santos; Mafalda Pinho; Carla S. Lopes

23 January 2023

10.1242/dmm.049693

RESEARCH ARTICLE

Lipopolysaccharide distinctively alters human microglia transcriptomes to resemble microglia from Alzheimer's disease mouse models

Jimena Monzón-Sandoval; Elena Burlacu; Devika Agarwal; Adam E. Handel; Liting Wei; John Davis; Sally A. Cowley; M. Zameel Cader; Caleb Webber

18 October 2022

10.1242/dmm.049349

REVIEW

Defining the unknowns for cell therapies in Parkinson's disease

Emma L. Lane; Mariah J. Lelos

27 September 2022

10.1242/dmm.049543

RESEARCH ARTICLE

Transcriptional targets of amyotrophic lateral sclerosis/frontotemporal dementia protein TDP-43 – meta-analysis and interactive graphical database

Maize C. Cao; Emma L. Scotter

13 September 2022

10.1242/dmm.049418

RESEARCH ARTICLE

Cerebellar neuronal dysfunction accompanies early motor symptoms in spinocerebellar ataxia type 3

Kristin Mayoral-Palarz; Andreia Neves-Carvalho; Sara Duarte-Silva; Daniela Monteiro-Fernandes; Patrícia Maciel; Kamran Khodakhah

05 August 2022

10.1242/dmm.049514

RESEARCH ARTICLE

Hsp40 overexpression in pacemaker neurons delays circadian dysfunction in a Drosophila model of Huntington's disease

Pavitra Prakash; Arpit Kumar Pradhan; Vasu Sheeba

28 June 2022

10.1242/dmm.049447

REVIEW

Deep phenotyping for precision medicine in Parkinson's disease

Ann-Kathrin Schalkamp; Nabila Rahman; Jimena Monzón-Sandoval; Cynthia Sandor

01 June 2022

10.1242/dmm.049376

RESEARCH ARTICLE

The GBA1 D409V mutation exacerbates synuclein pathology to differing extents in two alpha-synuclein models

Nicole K. Polinski; Terina N. Martinez; Sylvie Ramboz; Michael Sasner; Mark Herberth; Robert Switzer; Syed O. Ahmad; Lee J. Pelligrino; Sean W. Clark; Jacob N. Marcus; Sean M. Smith; Kuldip D. Dave; Mark A. Frasier

25 May 2022

10.1242/dmm.049192

RESOURCE ARTICLE

Temporal and spatially controlled APP transgene expression using Cre-dependent alleles

Emily J. Koller; Melissa Comstock; Jonathan C. Bean; Gabriel Escobedo, Jr; Kyung-Won Park; Joanna L. Jankowsky

13 May 2022

10.1242/dmm.049330

RESEARCH ARTICLE

TDP-43 promotes tau accumulation and selective neurotoxicity in bigenic Caenorhabditis elegans

Caitlin S. Latimer; Jade G. Stair; Joshua C. Hincks; Heather N. Currey; Thomas D. Bird; C. Dirk Keene; Brian C. Kraemer; Nicole F. Liachko

27 April 2022

10.1242/dmm.049323