DOI: 10.1523/jneurosci.4496-09.2010. Altered Intracellular Ca<sup>2+</sup>Homeostasis in Nerve Terminals of Severe Spinal Muscular Atrophy Mice

Altered Intracellular Ca2+Homeostasis in Nerve Terminals of Severe Spinal Muscular Atrophy Mice

10.1523/jneurosci.4496-09.2010

Crossref journal-article

Society for Neuroscience

The Journal of Neuroscience (393)

Abstract

Low levels of survival motor neuron (SMN) protein result in spinal muscular atrophy (SMA), a severe genetic disease characterized by motor impairment and premature lethality. Although SMN is a ubiquitous protein, motor neurons are much more vulnerable to low levels of SMN than other cells. To gain insight into the pathogenesis of SMA, we have compared synaptic function of motor terminals in wild-type and severe SMA mice at different ages and in two proximal muscles. Our results show that mutant muscle fibers fire normal action potentials and that multi-innervated terminals are functional. By studying the characteristics of the three main components of synaptic transmission in nerve terminals (spontaneous, evoked, and asynchronous release), we found that the kinetics of the postsynaptic potentials are slowed and evoked neurotransmitter release is decreased by ∼55%. In addition, asynchronous release is increased ∼300%, indicating an anomalous augmentation of intraterminal bulk Ca2+during repetitive stimulation. Together, these results show that the reduction of SMN affects synaptic maturation, evoked release, and regulation of intraterminal Ca2+levels.

Bibliography

Ruiz, R., CasaÃ±as, J. J., Torres-Benito, L., Cano, R., & Tabares, L. (2010). Altered Intracellular Ca2+Homeostasis in Nerve Terminals of Severe Spinal Muscular Atrophy Mice. The Journal of Neuroscience, 30(3), 849â857.

Authors 5

Rocío Ruiz (first)
Juan José Casañas (additional)
Laura Torres-Benito (additional)
Raquel Cano (additional)
Lucía Tabares (additional)

References 43 Referenced 141

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Dates

Type	When
Created	15 years, 7 months ago (Jan. 20, 2010, 3:23 p.m.)
Deposited	2 years, 4 months ago (April 13, 2023, 3:44 p.m.)
Indexed	2 months ago (June 26, 2025, 9:44 a.m.)
Issued	15 years, 7 months ago (Jan. 20, 2010)
Published	15 years, 7 months ago (Jan. 20, 2010)
Published Online	15 years, 7 months ago (Jan. 20, 2010)
Published Print	15 years, 7 months ago (Jan. 20, 2010)

Funders 0

None

BibTeX

@article{Ruiz_2010, title={Altered Intracellular Ca2+Homeostasis in Nerve Terminals of Severe Spinal Muscular Atrophy Mice}, volume={30}, ISSN={1529-2401}, url={http://dx.doi.org/10.1523/jneurosci.4496-09.2010}, DOI={10.1523/jneurosci.4496-09.2010}, number={3}, journal={The Journal of Neuroscience}, publisher={Society for Neuroscience}, author={Ruiz, Rocío and Casañas, Juan José and Torres-Benito, Laura and Cano, Raquel and Tabares, Lucía}, year={2010}, month=jan, pages={849–857} }

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