This is the cover of the DMM special issue showing primary cortical neuronal culture isolated from newborn mice.CREDIT Vito Antonio Baldassarro
A growing aging population
means that age-dependent neurodegenerative disorders such as Alzheimer's
disease, Parkinson's disease, Huntington's disease and amyotrophic lateral
sclerosis are affecting increasing numbers of people, making research into these
diseases more important than ever. As a crucial resource for researchers and to
showcase the vital research being done in the field, Disease Models &
Mechanisms
(DMM) is launching a new special collection - Neurodegeneration: from models to
mechanisms to therapies.
A special issue
of DMM is being published to launch the collection, guest edited by Aaron
Gitler (Stanford University) and James Shorter (the Perelman School of Medicine
at the University of Pennsylvania), both renowned specialists in
protein-misfolding neurodegenerative disorders. Our guest editors open the
issue with an editorial outlining historical models in neurodegeneration
research, and the potential for future breakthroughs using cutting-edge induced
pluripotent stem cell (iPSC) and 3D brain organoid models. They also discuss
new therapies such as the recent successful clinical trials for antisense
oligonucleotide (AO) therapy for spinal muscular atrophy, outline articles in
the special issue and highlight key neurodegeneration articles published
previously in DMM.
Following the Editorial,
DMM Founding Editor Huda Zoghbi describes in an interview how the cases she saw
as a clinical neurologist inspired her to move into the lab. A long-time
advocate of using model systems in human disease research and epitomising the
dedication and passion of the researchers in the field, Huda shares why it has
taken her 25 years to understand the biology of neurological disorders well
enough to start developing therapeutics. Another highlight of the special issue
is a poster by Edward Lee and colleagues providing a detailed visual
realisation of how impaired RNA metabolism can underlie the pathophysiology of
neurodegeneration, as well as discussing potential therapeutic interventions to
target these processes.
Spotlight on
neurodegenerative disorders
Amyotrophic lateral
sclerosis (ALS) is covered extensively in this special issue, beginning with a
review by Wim Robberecht and colleagues, which provides a comprehensive
discussion of model systems used to study ALS, from cell-based systems to fruit
flies to rodents. A research article from Javier Fernández-Ruiz and colleagues
describes a new spontaneous canine degenerative myelopathy model for ALS that
offers several advantages over traditional models relying on transgenic
overexpression. Further research articles discuss the molecular basis for
weight loss in neurodegenerative disorders with a focus on tyrosine receptor
kinase 3 (Kim et al., 2017), the role of autophagy dysregulation in ALS
(Wald-Altman et al., 2017), and a new Ranbp2 knockout mouse model for studying
the impairments in nucleocytoplasmic transport that can underlie
neurodegenerative diseases (Cho et al., 2017).
A second review article
presents a discussion by Gabriela Caraveo and colleagues on the current
evidence for dysregulation of calcium-dependent pathways in Parkinson's disease
(PD), and its implications in drug development. Research articles focusing on
PD describe how an analysis of blood samples from a large Turkish family
revealed complexin-1 as potential new blood biomarker in early PD risk
screening (Lahut et al., 2017), and how genetic mutations linked to PD lead to
stage-specific deregulation of the nucleolus (Evsyukov et al., 2017).
Less well-known
neurodegenerative disorders also feature in this special issue, including an in
vitro cellular model exploring Miller-Fisher syndrome (Rodella et al., 2017),
and a study into the role of the IKBKAP gene in hereditary sensory and
autonomic neuropathies (HSANs) of the peripheral nervous system (Chaverra et
al., 2017).
Remembering Susan
Lindquist
No compilation of current
neurodegeneration research would be complete without taking a moment to reflect
on the extraordinary contributions to the field by Susan Lindquist, who passed
away last year. Her legacy is clear in the number of articles in DMM authored
by, citing or mentioning Susan, including a poster co-authored with Julie
Valastyan that provides a snapshot of protein-level mechanisms implicated in
neurodegenerative and related disorders, and a moving tribute by Vivian Siegel.
This special collection is dedicated to her memory.
###
The primary aim of DMM is
to promote human health by inspiring collaboration between basic and clinical
researchers in translational science. All our articles are free to read, and we
are proud to launch this new special collection on neurodegeneration as part of
our ongoing focus on the use of model systems to better understand, diagnose
and treat human disease. Look out for new additions to this collection in
upcoming issues of the journal and sign up for email alerts on our website.
Disclaimer: AAAS and EurekAlert! are
not responsible for the accuracy of news releases posted to EurekAlert! by
contributing institutions or for the use of any information through the
EurekAlert system.
https://www.eurekalert.org/pub_releases/2017-05/tcob-dln042717.php
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