Calcium is reduced in presynaptic mitochondria of motor nerve terminals during neurotransmission in SMA mice.

ABSTRACT: Spinal muscular atrophy (SMA) is an autosomal recessive degenerative motor neuron disease characterized by symmetrical muscle weakness and atrophy of limb and trunk muscles being the most severe genetic disease in children. In SMA mouse models, motor nerve terminals display neurotransmitter release reduction, endocytosis decrease and mitochondria alterations. The relationship between these changes is, however, not well understood. In the present study, we investigated whether the endocytosis impairment could be related to the functional alteration of the presynaptic mitochondria during action potential (AP) firing. To this aim, we generated a Synaptophysin-pHluorin (SypHy) transgenic mouse, crossed it with Taiwanese SMA mice, and recorded exo- and endocytosis and mitochondria Ca2+ signalin

SUBMITTER: Lopez-Manzaneda M 

PROVIDER: S-EPMC8127408 | biostudies-literature | 2021 May

REPOSITORIES: biostudies-literature