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SHR Neuro Cancer Cardio Lipid Metab Microb
von Lewinski, F; Fuchs, J; Vanselow, BK; Keller, BU.
Low Ca2+ buffering in hypoglossal motoneurons of mutant SOD1 (G93A) mice.
Neurosci Lett. 2008; 445(3):224-228
Doi: 10.1016/j.neulet.2008.08.084
Web of Science
PubMed
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- Leading authors Med Uni Graz
- von Lewinski Friederike
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- Abstract:
- Mutations in the Cu/Zn superoxide dismutase (SOD1) gene are associated with amyotrophic lateral sclerosis (ALS), a fatal neurodegenerative disorder characterized by a selective degeneration of brainstem and spinal motoneurons. The pathomechanism of degeneration is still incompletely understood, but includes a disruption in cellular Ca2+ homeostasis. Here we report a quantitative microfluorometric analysis of the Ca2+ homeostasis in vulnerable hypoglossal motoneurons of neonatal mutant (G93A) SOD1 transgenic mice, a mouse model of human ALS. Ca2+ transient decay times (tau = 0.3 s), extrusion rates (gamma = 92 s(-1)) and exceptionally low intrinsic Ca2+ binding ratios (kappaS = 30) were found to be in the same range as compared to non-transgenic animals. Together with the previous observation of high Ca2+ binding ratios in ALS-resistant neurons (e.g. oculomotor), this supports the assumption that low Ca2+ buffering in vulnerable motoneurons represents a significant risk factor for degeneration. On the other hand, alterations in buffering properties by expression of mutant SOD1 are unlikely to be involved in disease initiation.
- Find related publications in this database (using NLM MeSH Indexing)
- Animals -
- Animals, Newborn -
- Brain Stem - cytology
- Calcium - metabolism
- Homeostasis - genetics
- Humans -
- In Vitro Techniques -
- Mice -
- Mice, Transgenic -
- Motor Neurons - metabolism
- Mutation -
- Superoxide Dismutase - genetics
- Time Factors -
- Find related publications in this database (Keywords)
- ALS
- Calcium
- Buffer
- Mitochondria
- SOD1
- Motoneuron