期刊
EMBO REPORTS
卷 21, 期 8, 页码 -出版社
WILEY
DOI: 10.15252/embr.201948882
关键词
fragile X syndrome; mitochondria; synapses; translation
资金
- NCN grant SONATA BIS [2014/14/E/NZ3/00375]
- National Science Centre [OPUS6 2013/11/B/NZ3/00974, OPUS10 2015/19/B/NZ3/03272]
- ministerial funds for science within Ideas Plus program [000263]
- International Research Agendas programme of the Foundation for Polish Science [MAB/2017/2]
- European Union under the European Regional Development Fund
- Centre for Preclinical Research and Technology (CePT)
- European Regional Development Fund and Innovative Economy
- National Cohesion Strategy of Poland
Synapses are the regions of the neuron that enable the transmission and propagation of action potentials on the cost of high energy consumption and elevated demand for mitochondrial ATP production. The rapid changes in local energetic requirements at dendritic spines imply the role of mitochondria in the maintenance of their homeostasis. Using global proteomic analysis supported with complementary experimental approaches, we show that an essential pool of mitochondrial proteins is locally produced at the synapse indicating that mitochondrial protein biogenesis takes place locally to maintain functional mitochondria in axons and dendrites. Furthermore, we show that stimulation of synaptoneurosomes induces the local synthesis of mitochondrial proteins that are transported to the mitochondria and incorporated into the protein supercomplexes of the respiratory chain. Importantly, in a mouse model of fragile X syndrome,Fmr1KO mice, a common disease associated with dysregulation of synaptic protein synthesis, we observed altered morphology and respiration rates of synaptic mitochondria. That indicates that the local production of mitochondrial proteins plays an essential role in synaptic functions.
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