A G309D-Pink1 knock-in mouse model of the PARK6-form of Parkinson's disease

• Antragstellerin: Dr. Suzana Gispert-Sánchez
• Fachliche Zuordnung: Molekulare Biologie und Physiologie von Nerven- und Gliazellen
• Förderung: Förderung von 2005 bis 2007
• Projektkennung: Deutsche Forschungsgemeinschaft (DFG) - Projektnummer 18857684

• Erstellungsjahr: 2009

Zusammenfassung der Projektergebnisse

The G309D-Pinkl knock-in and a Pinkl knock-out (KO) mouse were generated. The KO mouse showed adult onset and progression of impaired spontaneous movement, together with brain mitochondrial and synaptic dysfunction in the absence of neurodegeneration. Murine embryonal fibroblasts did not show the typical features of PARK6 patient fibroblasts. The project results underline the relevance of Pinkl for mitochondrial bioenergetics.

Projektbezogene Publikationen (Auswahl)

• Abahuni N, Gispert S, Bauer P, Riess O, Krüger R, Becker T, Auburger G. Mitochondrial translation initiation factor 3 gene polymorphism associated with Parkinson's disease. Neurosci Lett. 2007 Mar 6;414(2): 126-9.
  
  
• Exner N, Treske B, Paquet D, Holmström K, Schiesling C, Gispert 5, Carballo-Carbajal I, Berg D, Hoepken HH, Gasser T, Krüger R, Winklhofer KF, Vogel F, Reichert AS, Auburger G, Kahle PJ, Schmid B, Haass C. Loss-of-function ofhuman PINKl results in mitochondrial pathology and can be rescued by parkin. J Neurosci. 2007 Nov 7:27f45):12413-8.
  
  
• Hoepken HH, Gispert 5, Morales B, Wingerter O, Del Turco D, Mülsch A, Nussbaum RL, Müller K, Dröse S, Brandt U, Deller T, Wirth B, Kudin AP, Kunz WS, Auburger G. Mitochondrial dysfunction, peroxidation damage and changes in glutathione metabolism in PARK6. Neurobiol Dis. 2007 Feb;25(2):401-ll. Epub 2006 Nov 30.
  
  
• Hoepken HH, Gispert S, Azizov M, Klinkenberg M, Ricciardi F, Kurz A, Morales-Gordo B, Bonin M, Riess O, Gasser T, Kögel D, Steinmetz H, Auburger G. Parkinson patient fibroblasts show increased alpha-synuclein expression. Exp Neurol. 2008 Aug;212(2):307- 13. Epub 2008 Apr 16.