Issues
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Cover image
Cover Image
Cover: Primary cortical neuronal culture isolated from newborn Tg2576 mice, expressing the human amyloid precursor protein (APP, isoform 695) carrying the Swedish mutation (APPswe; KM670/671NL). Cells were allowed to mature in vitro for 8 days and then characterized for culture composition by immunocytochemistry for neural (β-III-tubulin; green) and astroglial (GFAP; red) markers. See article by Baldassarro et al. on page 671. Cover image by Vito Antonio Baldassarro is licensed under a Creative Commons Attribution 4.0 International licence.
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SPECIAL COLLECTION: NEURODEGENERATION: FROM MODELS TO MECHANISMS TO THERAPIES
EDITORIAL
Neurodegenerative disease: models, mechanisms, and a new hope
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
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
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.
REVIEWS
The role of Ca2+ signaling in Parkinson's disease
Summary: This Review article discusses the important roles that calcium plays in the pathogenesis of Parkinson's disease.
Modelling amyotrophic lateral sclerosis: progress and possibilities
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
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.
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
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.
The tyrosine kinase receptor Tyro3 enhances lifespan and neuropeptide Y (Npy) neuron survival in the mouse anorexia (anx) mutation
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.
Schwann cells are activated by ATP released from neurons in an in vitro cellular model of Miller Fisher syndrome
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.
The familial dysautonomia disease gene IKBKAP is required in the developing and adult mouse central nervous system
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.
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
Summary: Complexin 1 is a prodromal biomarker and risk factor for REM sleep behavior disorder and PARK4-associated Parkinson's disease.
Genetic mutations linked to Parkinson's disease differentially control nucleolar activity in pre-symptomatic mouse models
Summary: Genetic mutations linked to Parkinson's disease lead to stage-specific deregulation of the nucleolus, a major integrator of the cellular stress response.
A differential autophagy-dependent response to DNA double-strand breaks in bone marrow mesenchymal stem cells from sporadic ALS patients
Summary: A novel endogenous disease mechanism in cells from ALS patients after NCS-induced DNA damage.
Cellular levels of Grb2 and cytoskeleton stability are correlated in a neurodegenerative scenario
Summary: Grb2 has a unique role in protecting the cytoskeletal architecture in AD-like conditions, offering a potential new strategy for controlling neurodegeneration.
Vulnerability of primary neurons derived from Tg2576 Alzheimer mice to oxygen and glucose deprivation: role of intraneuronal amyloid-β accumulation and astrocytes
Summary: In vitro systems derived from AD mice can be used to investigate the vulnerability of AD neurons to different neurotoxic challenges, including oxygen glucose deprivation.
Virtual Meeting - Developmental Disorders: From Mechanism to Treatment
A collaboration between the Development and Disease Models & Mechanisms journal teams, this virtual Meeting will unite developmental biologists, human geneticists and clinical researchers to focus on building bridges from bench to clinic. Register your interest and find out more.
High-dose vitamin B1 therapy prevents the development of experimental fatty liver driven by overnutrition
Vitamin B1 is shown to prevent nutrition-induced fatty liver in our current Editor’s choice by Hay Davir and his colleagues, when they use sheep as a large-animal model for studying the disease.
Find out more in an interview with the paper's first authors, Mugagga Kalyesubula and Ramgopal Mopuri.
Upcoming grant deadlines
Grants awarded by The Company of Biologists help scientists travel, attend events and host sustainable activities. Make a note of the upcoming application deadlines and find out more about the grants on offer:
DMM Conference Travel Grants
17 May 2021
Sustainable Conferencing Grants
17 May 2021
Travelling Fellowships
31 May 2021
Scientific Meeting Grants
4 June 2021
Call for papers - The RAS Pathway: Diseases, Therapeutics and Beyond
Our upcoming special issue is welcoming submissions until 3 May 2021. Guest-edited by Donita Brady (Perelman School of Medicine at the University of Pennsylvania, USA) and Arvin Dar (Icahn School of Medicine at Mount Sinai, USA), the issue will focus on targeting the RAS pathway.