Is the expression of [-G93A(+)] human SOD1 a model to study neurodegenerations?

Autor: Monica Averna, Marco Pedrazzi, Edon Melloni, R. De Tullio, Sandro Pontremoli, F. Salamino, Roberto Stifanese
Jazyk: angličtina
Rok vydání: 2011
Předmět:
Zdroj: Journal of Biological Research, Vol 84, Iss 1 (2011)
ISSN: 2284-0230
1826-8838
Popis: To relate the alterations occurring in neurodegenerations with Ca2+ homeostasis dysregulation, we analyzed the functional properties of the Ca2+-dependent calpain/calpastatin system in neuronal cells of transgenic mice overexpressing human mutated -G93A(+) SOD1. Motor cortex and spinal cord lower segments from transgenic mice show a very large increase in free Ca2+ ions and evident calpain activation, identified onthe basis of its consumption and substrates digestion. Changes in calpastatin intracellular localization and calpain conformation state further support these observations. Moreover, calpastatin is significantly expressed, counteracting its calpain-mediated degradation. Thus, the calpain /calpastatin system may be a target for new therapeutic approaches to neurodegenerative diseases.
Databáze: OpenAIRE
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