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Antiglycative Activity and RAGE Expression in Rett Syndrome.
Valeria Cordone1, Alessandra Pecorelli2, Mascia Benedusi3
1Department of Life, Health and Environmental Sciences, University of L'Aquila, Via Vetoio, 67100 L'Aquila, Italy. vcordone@unite.it.
Cells
|February 21, 2019
Summary
Rett syndrome (RTT) involves altered defenses against methylglyoxal (MG), a toxic compound. RTT cells show increased glyoxalase 2 activity but heightened cell death when exposed to MG.
Area of Science:
- Neuroscience
- Biochemistry
- Cell Biology
Background:
- Rett syndrome (RTT) pathogenesis is linked to oxidative stress and inflammation (OxInflammation).
- Methylglyoxal (MG), a cytotoxic byproduct, drives advanced glycation end product (AGE) formation, impacting neurological diseases via RAGE.
- Understanding cellular defense mechanisms against MG is crucial for RTT research.
Purpose of the Study:
- To compare RTT patient fibroblasts with healthy controls regarding methylglyoxal (MG) metabolism and stress response.
- To investigate the glyoxalase (GLO) system activity and MG-dependent damage in RTT cells.
- To evaluate RAGE levels and cellular sensitivity to exogenous MG in RTT.
Main Methods:
- Fibroblast cultures from RTT patients (N=6) and healthy controls (CTR, N=6) were used.
- Gene/protein expression, glyoxalase (GLO) enzymatic activities, and MG-dependent damage were assessed.
- Cells were analyzed under basal and methylglyoxal (MG)-challenged conditions.
Main Results:
- RTT fibroblasts exhibited altered glyoxalase system activity, specifically increased GLO2 activity, maintaining normal MG-dependent damage levels.
- RTT cells showed increased susceptibility to cell death upon exogenous MG treatment compared to CTR.
- RTT cells displayed lower RAGE levels than CTR, with no significant change after MG treatment, suggesting an adaptive response.
Conclusions:
- Rett syndrome is associated with a modified defense system against dicarbonyl stress, beyond OxInflammation.
- RTT cells exhibit an altered stress response to pro-glycating insults, including increased vulnerability to methylglyoxal.
- The findings suggest complex cellular adaptations in RTT impacting responses to metabolic stress.
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