Wyniki wyszukiwania

Wyszukiwano:
w słowach kluczowych: disease model

Sortuj według:

Ogranicz wyniki do:

Induced pluripotent stem cells in modeling and cell-based therapy of amyotrophic lateral sclerosis

100%

Csobonyeiova M., Polak S., Nicodemou A., Danisovic L.

Journal of Physiology and Pharmacology

2017

tom 68

nr 5

Alpha-synuclein A30P point-mutation generates age-dependent nigrostriatal deficiency in mice

84%

Plaas M., Karis A., Innos J., Rebane E., Baekelandt V., Vaarmann A., Luuk H., Vasar E., Koks S.

Journal of Physiology and Pharmacology

2008

tom 59

nr 2

Lewy bodies are mainly composed of alpha-synuclein (SNCA) and specific mutations in SNCA gene are related to familial forms of Parkinson's disease (PD). The purpose of our study was to generate a mouse line with A30P knock-in point mutation in SNCA gene and to test if a single point-mutation is able to turn otherwise normal SNCA into a toxic form. The behavioral profile of SNCA A30P mice was followed for 16 months. Generally, these mice are healthy and viable without any obvious abnormalities. Starting from the age of 13 months mice developed a significant deficit in motor performance tests related to nigrostriatal function (ink-test and beam walk). In other tests (motility boxes, rotarod) mice continuously performed normally. Moreover, SNCA A30P mice expressed the altered sensitivity to VMAT2 inhibitor reserpine, possibly reflecting a functional deficiency of dopamine. Indeed, mice at 15 months of age had significantly reduced levels of dopamine and its major metabolite DOPAC in the striatum, and reduced levels of dopamine in the mesolimbic system. The present study confirms that SNCA plays an important role in the development of PD and an insertion of a single point mutation is sufficient to generate age-related decline in specific motor performance. The generated mouse line has a potential to become a model for PD with comparable time course and phenotype.

Ograniczanie wyników

2 Journal of Physiology and Pharmacology

1 Baekelandt V.

1 Csobonyeiova M.

1 Danisovic L.

1 Innos J.

1 Karis A.

1 Koks S.

1 Luuk H.

1 Nicodemou A.

1 Plaas M.

1 Polak S.

1 Rebane E.

1 Vaarmann A.

1 Vasar E.

1 2017

1 2008

Wyniki wyszukiwania

Induced pluripotent stem cells in modeling and cell-based therapy of amyotrophic lateral sclerosis

Alpha-synuclein A30P point-mutation generates age-dependent nigrostriatal deficiency in mice