Downstream mechanisms triggered by mitochondrial dysfunction in the basal ganglia: From experimental models to neurodegenerative diseases
| Autor: | Paolo Gubellini, Barbara Picconi, Massimiliano Di Filippo, Paolo Calabresi |
|---|---|
| Přispěvatelé: | Institut de Biologie du Développement de Marseille (IBDM), Aix Marseille Université (AMU)-Centre National de la Recherche Scientifique (CNRS), Fondazione Santa Lucia [IRCCS], Clinical and Behavioral Neurology [IRCCS Santa Lucia], Ospedale 'Santa Maria della Misericordia' = University Hospital 'Santa Maria della Misericordia' |
| Jazyk: | angličtina |
| Rok vydání: | 2010 |
| Předmět: |
MESH: Cell Death
Huntingtin Parkinson's disease striatum Gene mutation Parkin Basal Ganglia 3-nitropropionic acid MESH: Neurodegenerative Diseases MESH: Basal Ganglia rotenone 0302 clinical medicine Basal ganglia MESH: Animals Basal Ganglia/*metabolism/physiopathology parkin Basal ganglia disease 0303 health sciences Cell Death Neurodegenerative Diseases Parkinson Disease Parkinson Disease/metabolism 3. Good health Mitochondria Huntington Disease Biochemistry mitochondrial fusion Molecular Medicine Huntington Disease/metabolism dopamine MESH: Mitochondria huntingtin PINK1 [SDV.BC]Life Sciences [q-bio]/Cellular Biology Biology Huntington's chorea 03 medical and health sciences medicine Neurodegenerative Diseases/*metabolism Animals Molecular Biology long-term potentiation 030304 developmental biology Mitochondria/*metabolism Animal medicine.disease MESH: Huntington Disease Disease Models Animal Disease Models DNAJA3 MESH: Disease Models Animal Neuroscience 030217 neurology & neurosurgery MESH: Parkinson Disease |
| Zdroj: | Biochimica et Biophysica Acta (BBA)-Molecular Basis of Disease; Vol 1802 Biochimica et Biophysica Acta (BBA)-Molecular Basis of Disease Biochimica et Biophysica Acta-Molecular Basis of Disease Biochimica et Biophysica Acta-Molecular Basis of Disease, Elsevier, 2009, 1802 (1), pp.151. ⟨10.1016/j.bbadis.2009.08.001⟩ BBA-Biochimica et Biophysica Acta BBA-Biochimica et Biophysica Acta, Elsevier, 2010, 1802 (1), pp.151-61. ⟨10.1016/j.bbadis.2009.08.001⟩ BBA-Biochimica et Biophysica Acta, 2010, 1802 (1), pp.151-61. ⟨10.1016/j.bbadis.2009.08.001⟩ |
| ISSN: | 0925-4439 0006-3002 |
| DOI: | 10.1016/j.bbadis.2009.08.001 |
| Popis: | International audience; Mitochondrial dysfunctions have been implicated in the cellular processes underlying several neurodegenerative disorders affecting the basal ganglia. These include Huntington's chorea and Parkinson's disease, two highly debilitating motor disorders for which recent research has also involved gene mutation linked to mitochondrial deficits. Experimental models of basal ganglia diseases have been developed by using toxins able to disrupt mitochondrial function: these molecules act by selectively inhibiting mitochondrial respiratory complexes, uncoupling cellular respiration. This in turn leads to oxidative stress and energy deficit that trigger critical downstream mechanisms, ultimately resulting in neuronal vulnerability and loss. Here we review the molecular and cellular downstream effects triggered by mitochondrial dysfunction, and the different experimental models that are obtained by the administration of selective mitochondrial toxins or by the expression of mutant genes. |
| Databáze: | OpenAIRE |
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