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 |
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Jazyk: | angličtina |
Rok vydání: | 2011 |
Předmět: |
Genetically modified mouse
biology Chemistry Intracellular localization Biochemistry (medical) SOD1 Calpain Ca2+-dependent proteolysis calpain calpastatin NOS ALS Plant Science Spinal cord Molecular biology General Biochemistry Genetics and Molecular Biology medicine.anatomical_structure lcsh:Biology (General) biology.protein medicine lcsh:QH301-705.5 Homeostasis Calpastatin |
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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