Modelovanie ochorenia a štúdium regeneračných procesov v Huntingtonovej chorobe a ALS in vivo
| Autor: | Hruška-Plocháň, Marián |
|---|---|
| Jazyk: | angličtina |
| Rok vydání: | 2013 |
| Předmět: |
transgenesis
transplantace protein aggregates lentiviral vectors lidské fetální neurální kmenové buňky buňková terapie spinal cord differentiation traumatic spinal cord injury (SCI) regeneration of the spinal cord immunosuppression Huntington's disease miniaturní prase minipig gad mutant mouse human fetal neural stem cells motoneuron ubiquitin proteázomový systém takrolimus transgeneze R6 SOD1 Amyotrofická laterální skleróza 2 myš cell-replacement therapy ubiquitin proteasome system lentivirové vektory 2 HD mouse imunosuprese Huntingtonova choroba UCHL1 mícha huntingtin striatum regenerace míchy traumatické poškození míchy gad myš tacrolimus transplantation diferenciace proteinové agregáty ALS |
| Popis: | Neurological disorders affect more than 14% of the population worldwide and together with traumatic brain and spinal cord injuries represent major health, public and economic burden of the society. Incidence of inherited and idiopathic neurodegenerative disorders and acute CNS injuries is growing globally while neuroscience society is being challenged by numerous unanswered questions. Therefore, research of the CNS disorders is essential. Since animal models of the CNS diseases and injuries represent the key step in the conversion of the basic research to the clinics, we focused our work on generation of new animal models and on their use in pre-clinical research. We generated and characterized transgenic minipig model of Huntington's disease (HD) which represents the only successful establishment of a transgenic model of HD in minipig which should be valuable for testing of long term safety of HD therapeutics. Next, we crossed the well characterized R6/2 mouse HD model with the gad mouse model which lacks the expression of UCHL1 which led to results that support the theory of "protective" role of mutant huntingtin aggregates and suggest that UCHL1 function(s) may be affected in HD disturbing certain branches of Ubiquitin Proteasome System. Traumatic spinal cord injury and Amyotrophic Lateral... |
| Databáze: | OpenAIRE |
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