Browsing by Author "Saukkonen, Anni"

Amyotrophic lateral sclerosis (ALS) is a progressive fatal neurodegenerative disease affecting motor neurons. It finally leads to the malfunction of the respiratory muscles and death after 1-3 years of diagnosis. Sporadic cases of ALS cover 90-95% of all patients and familial 5-10% respectively. The onset of the disease is usually between age of 40 and 60 and the worldwide incidence is considered to be 1-2/100000. Currently discovered cerebral dopamine neurotrophic factor, CDNF, has showed neuroprotective effects on Parkinson's disease model. What is more, it is known that CDNF is expressed in the muscles of mice and one of its' main functions is to protect cells from ER-stress, one of the pathological mechanisms in ALS. Hence, it is rational to study the effects of CDNF in ALS mouse model. Treatment options are needed, since there is only one approved treatment for ALS, anti-glutaminergic rilutzole. The aim of this study was to find out whether CDNF shows neuroprotective effects in SOD1-mice e.g. by measuring the changes in motor function with different behavioral tests. More over, the distribution of CDNF after intrathecal ventricle injection was studied using immunohistochemical and radioactive labeling methods. The hypothesis was that CDNF is distributed through the cerebrospinal fluid into the spinal cord and muscles in the limbs and shows neuroprotective effects in this SOD1 mouse model.