Hereditary cerebellar ataxias are severe diseases for which therapy is currently not sufficiently effective. One of the possible therapeutic approaches could be neurotransplantation. Lurcher mutant mice are a natural model of olivocerebellar degeneration representing a tool to investigate its pathogenesis as well as experimental therapies for hereditary cerebellar ataxias. The effect of intracerebellar transplantation of embryonic cerebellar solid tissue or cell suspension on motor performance in adult Lurcher mutant and healthy wild-type mice was studied. Brain-derived neurotrophic factor level was measured in the graft and adult cerebellar tissue. Gait analysis and rotarod, horizontal wire, and wooden beam tests were carried out 2 or 6 months after the transplantation. Higher level of the brain-derived neurotrophic factor was found in the Lurcher cerebellum than in the embryonic and adult wild-type tissue. A mild improvement of gait parameters was found in graft-treated Lurcher mice. The effect was more marked in cell suspension grafts than in solid transplants and after the longer period than after the short one. Lurcher mice treated with cell suspension and examined 6 months later had a longer hind paw stride (4.11 vs. 3.73 mm, P < 0.05) and higher swing speed for both forepaws (52.46 vs. 32.79 cm/s, P < 0.01) and hind paws (63.46 vs. 43.67 cm/s, P < 0.001) than controls. On the other hand, classical motor tests were not capable of detecting clearly the change in the motor performance. No strong long-lasting negative effect of the transplantation was seen in wild-type mice, suggesting that the treatment has no harmful impact on the healthy cerebellum.
- MeSH
- časové faktory MeSH
- chůze (způsob) MeSH
- metoda rotující tyčky MeSH
- mozeček embryologie metabolismus transplantace MeSH
- mozkový neurotrofický faktor metabolismus MeSH
- multisystémová atrofie patofyziologie terapie MeSH
- myši - mutanty neurologické MeSH
- myši inbrední C57BL MeSH
- myši inbrední CBA MeSH
- myši transgenní MeSH
- pohybová aktivita MeSH
- spinocerebelární degenerace patofyziologie terapie MeSH
- transplantace fetální tkáně metody MeSH
- transplantace mozkové tkáně metody MeSH
- výsledek terapie MeSH
- zelené fluorescenční proteiny genetika metabolismus MeSH
- zvířata MeSH
- Check Tag
- zvířata MeSH
- Publikační typ
- časopisecké články MeSH
- práce podpořená grantem MeSH
- MeSH
- amyotrofická laterální skleróza diagnóza farmakoterapie klasifikace MeSH
- hepatolentikulární degenerace diagnóza etiologie farmakoterapie MeSH
- lidé MeSH
- multisystémová atrofie diagnóza farmakoterapie MeSH
- neurodegenerativní nemoci * diagnóza farmakoterapie MeSH
- spastická paraparéza diagnóza farmakoterapie komplikace MeSH
- spinocerebelární degenerace diagnóza etiologie farmakoterapie terapie MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- přehledy MeSH
- MeSH
- demence diagnóza etiologie terapie MeSH
- hepatolentikulární degenerace diagnóza terapie MeSH
- lidé MeSH
- motorické poruchy diagnóza psychologie terapie MeSH
- neurodegenerativní nemoci * diagnóza patofyziologie terapie MeSH
- Parkinsonova nemoc diagnóza etiologie MeSH
- parkinsonské poruchy diagnóza terapie MeSH
- polékové dyskineze diagnóza terapie MeSH
- spinocerebelární degenerace diagnóza terapie MeSH
- Tourettův syndrom diagnóza terapie MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- přehledy MeSH