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Aversive and non-aversive memory impairment in the mucopolysaccharidosis II mouse model
Amanda Stapenhorst Azambuja1,2, Lilian Correa1,2, Bernardo Pappi Gabiatti2
1Programa de Pós-Graduação em Ciências Biológicas: Fisiologia-UFRGS, Porto Alegre, Brazil.
Abstract:
Hunter syndrome (MPS II, OMIM 309900) is a lysosomal storage disorder due to deficient iduronate sulphatase activity. Patients present multiple cognitive alterations, and the aim of this work was to verify if MPS II mice also present some progressive cognitive alterations. For that, MPS II mice from 2 to 6 months of age were submitted to repeated open field and inhibitory avoidance tests to evaluate memory parameters. MPS II mice presented impaired memory at 6 months evaluated by open field test. They also performed poorly in the inhibitory avoidance test from 4 months. We conclude that MPS II mice develop cognitive alterations as the disease progresses. These tests can be used in the future to study the efficacy of therapeutic approaches in the central nervous system.
Insights
Hunter syndrome (Mucopolysaccharidosis II) causes cognitive decline in patients. This study found that MPS II mice also exhibit progressive memory impairments, validating their use for testing new therapies.
Area of Science:
- Neuroscience
- Genetics
- Biochemistry
Background:
- Hunter syndrome (Mucopolysaccharidosis II) is a lysosomal storage disorder.
- It results from deficient iduronate sulphatase enzyme activity.
- Patients often experience cognitive alterations.
Purpose of the Study:
- To investigate progressive cognitive alterations in MPS II mouse models.
- To determine if MPS II mice exhibit memory deficits comparable to human patients.
- To establish the utility of these mice for evaluating central nervous system therapies.
Main Methods:
- MPS II mice aged 2 to 6 months were used.
- Behavioral assessments included the open field test and inhibitory avoidance test.
- Memory parameters were evaluated using these established behavioral tests.
Main Results:
- MPS II mice showed impaired memory in the open field test at 6 months of age.
- Poor performance in the inhibitory avoidance test was observed from 4 months onwards.
- These findings indicate progressive cognitive deficits in the MPS II mouse model.
Conclusions:
- MPS II mice develop progressive cognitive alterations mirroring human disease.
- The observed memory impairments validate the MPS II mouse model for research.
- These behavioral tests can assess the efficacy of future therapeutic interventions for MPS II.
