Mitochondrial dysfunction occurs in many late-onset neurodegenerative disorders (including Alzheimer's disease) and in developmental neurodegenerative disorders, such as Leigh syndrome, which is caused by mutations in the electron transport chain (ETC). But how does mitochondrial dysfunction cause neurodegeneration? Mast and co-workers perturbed the ETC in the Drosophila retina and now report that reactive oxygen species(ROS) overproduction in the photoreceptor cell body causes synaptic degeneration (see p. 2669). Mutations in the ETC component succinate dehydrogenase do not affect the early stages of photoreceptor development but cause degeneration of the photoreceptor synapses and cell bodies in late pupal and adult animals. ROS production, not energy depletion, causes this synaptic degeneration. Furthermore, ROS production in the cell body is sufficient to cause synaptic degeneration. These results establish the first animal model for Leigh syndrome and, more generally, suggest that excessive ROS production might cause some of the pathological changes seen in other neurodegenerative disorders.
Fly view of ROS and neurodegenerative disease
Fly view of ROS and neurodegenerative disease. Development 1 August 2008; 135 (15): e1506. doi:
Download citation file:
Advertisement
Cited by
Call for papers: Uncovering Developmental Diversity
Development invites you to submit your latest research to our upcoming special issue: Uncovering Developmental Diversity. This issue will be coordinated by our academic Editor Cassandra Extavour (Harvard University, USA) alongside two Guest Editors: Liam Dolan (Gregor Mendel Institute of Molecular Plant Biology, Austria) and Karen Sears (University of California Los Angeles, USA).
Choose Development in 2024
In this Editorial, Development Editor-in-Chief James Briscoe and Executive Editor Katherine Brown explain how you support your community by publishing in Development and how the journal champions serious science, community connections and progressive publishing.
Journal Meeting: From Stem Cells to Human Development
Register now for the 2024 Development Journal Meeting From Stem Cells to Human Development. Early-bird registration deadline: 3 May. Abstract submission deadline: 21 June.
Pluripotency of a founding field: rebranding developmental biology
This collaborative Perspective, the result of a workshop held in 2023, proposes a set of community actions to increase the visibility of the developmental biology field. The authors make recommendations for new funding streams, frameworks for collaborations and mechanisms by which members of the community can promote themselves and their research.
Read & Publish Open Access publishing: what authors say
We have had great feedback from authors who have benefitted from our Read & Publish agreement with their institution and have been able to publish Open Access with us without paying an APC. Read what they had to say.