Kuzniewska 2020 EMBO Rep

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Kuzniewska B, Cysewski D, Wasilewski M, Sakowska P, Milek J, Kulinski TM, Winiarski M, Kozielewicz P, Knapska E, Dadlez M, Chacinska A, Dziembowski A, Dziembowska M (2020) Mitochondrial protein biogenesis in the synapse is supported by local translation. EMBO Rep [Epub ahead of print].

» PMID: 32558077 Open Access

Kuzniewska Bozena, Cysewski Dominik, Wasilewski Michal, Sakowska Paulina, Milek Jacek, Kulinski Tomasz M, Winiarski Maciej, Kozielewicz Pawel, Knapska Ewelina, Dadlez Michal, Chacinska Agnieszka, Dziembowski Andrzej, Dziembowska Magdalena (2020) EMBO Rep

Abstract: 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, Fmr1 KO 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.

Keywords: Fragile X syndrome, Mitochondria Synapses, Translation Bioblast editor: Plangger M

Labels: MiParea: Respiration, mt-Biogenesis;mt-density 

Organism: Human, Mouse  Tissue;cell: Nervous system, HEK  Preparation: Permeabilized cells 

Coupling state: LEAK, ET  Pathway: N, S  HRR: Oxygraph-2k