| Literature DB >> 27324790 |
André Felipe Rodrigues1, Helena Biasibetti1, Bruna Stela Zanotto1, Eduardo Farias Sanches1, Felipe Schmitz1, Vinícius Tejada Nunes2, Paula Pierozan1, Vanusa Manfredini2, Débora Delwing Dal Magro3, Carlos Alexandre Netto1,4, Angela T S Wyse5,6.
Abstract
Classical galactosemia is an inborn error of carbohydrate metabolism in which patients accumulate high concentration of galactose in the brain. The most common treatment is a galactose-restricted diet. However, even treated patients develop several complications. One of the most common symptoms is motor coordination impairment, including affected gait, balance, and speech, as well as tremor and ataxia. In the present study, we investigated the effects of intracerebroventricular galactose administration on motor coordination, as well as on histological and biochemical parameters in cerebellum of adult rats. Wistar rats received 5 μL of galactose (4 mM) or saline by intracerebroventricular injection. The animals performed the beam walking test at 1 and 24 h after galactose administration. Histological and biochemical parameters were performed 24 h after the injections. The results showed motor coordination impairment at 24 h after galactose injection. Galactose also decreased the number of cells in the molecular and granular layers of the cerebellum. The immunohistochemistry results suggest that the cell types lost by galactose are neurons and astrocytes in the spinocerebellum and neurons in the cerebrocerebellum. Galactose increased active caspase-3 immunocontent and acetylcholinesterase activity, decreased acetylcholinesterase immunocontent, glutathione, and BDNF levels, as well as caused protein and DNA damage. Our results suggest that galactose induces histological and biochemical changes in cerebellum, which can be associated with motor coordination impairment.Entities:
Keywords: Acetylcholinesterase; Apoptosis; BDNF; Caspase-3; Cell damage; Galactose; Motor coordination
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Year: 2016 PMID: 27324790 DOI: 10.1007/s12035-016-9981-4
Source DB: PubMed Journal: Mol Neurobiol ISSN: 0893-7648 Impact factor: 5.590