Session Information
Date: Tuesday, June 21, 2016
Session Title: Rare genetic and metabolic diseases
Session Time: 12:30pm-2:00pm
Location: Exhibit Hall located in Hall B, Level 2
Objective: The present study was performed to investigate the effect of galactose (GAL) (at concentration in the brain, found in classical galactosemia) on motor coordination as well as histological and biochemical parameters in the rat cerebellum.
Background: Classical galactosemia is marked by tissue accumulation of GAL. However, GAL dietary restriction is not able to prevent patients from symptoms like impaired motor coordination, which starts to develop early in life. Furthermore, GAL is endogenously produced, leading to the believe that this metabolite participates in the toxicity of classical galactosemia. The cerebellum is well known to play a key role in movement control, and another common feature is cerebellar atrophy in classical galactosemia.
Methods: GAL (4mM) was injected intracerebroventricularly in 60-days-old Wistar rats, control animals received saline. The volume of the solution (saline or GAL) administered was 5 μl. Motor coordination was assessed 1h and 24h after injection by using the beam walking test. In the cerebellum, histopathological (hematoxylin/eosin and immunohistochemistry) and biochemical (active caspase-3 and BDNF levels) parameters were performed 24h after injections.
Results: Motor coordination impairment was found 24h after GAL injection. GAL also decreased the number of cells in the molecular and granular layer of the spinocerebellum, while GAL reduced the number of cells in the molecular layer of the cerebrocerebellum. Immunohistochemistry analyses suggested that the cell types lost were neurons and astrocytes in the spinocerebellum and neurons in the cerebrocerebellum. Furthermore, the results showed that galactose increased the immunocontent of active caspase-3 and decreased total BDNF immunocontent.
Conclusions: The results support that intracerebroventricular GAL administration causes motor impairment due to cell loss in the cerebellum which is at least in part caused by apoptosis. The findings also contribute to better understand the neurotoxic mechanisms behind the neuropathology of classical galactosemia.
To cite this abstract in AMA style:
A.F. Rodrigues, B.S. Zanotto, H. Biasibetti, P. Pierozan, F. Schmitz, E.F. Sanches, D. Delwing Dal Magro, C.A. Netto, A.T.S. Wyse. Intracerebroventricular D-galactose injection provokes motor coordination impairment and cerebellar damage in Wistar rats [abstract]. Mov Disord. 2016; 31 (suppl 2). https://www.mdsabstracts.org/abstract/intracerebroventricular-d-galactose-injection-provokes-motor-coordination-impairment-and-cerebellar-damage-in-wistar-rats/. Accessed June 14, 2025.« Back to 2016 International Congress
MDS Abstracts - https://www.mdsabstracts.org/abstract/intracerebroventricular-d-galactose-injection-provokes-motor-coordination-impairment-and-cerebellar-damage-in-wistar-rats/