Aversive and non-aversive memory impairment in the mucopolysaccharidosis II mouse model

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.

Mucopolysaccharidosis Type II: One Hundred Years of Research, Diagnosis, and Treatment

“…Despite the X-ray imaging confirming progressive skeletal impairment starting as early as 2 months, the behavioral analysis only highlighted a reduced activity and performance in the 8-month-old mice [225]. The behavioral impairment was also confirmed by another study using the open-field and inhibitory avoidance tests, but evidencing defects starting as early as 6 months [226]. To better understand the neurological pathology, Salvalaio and colleagues carried out an RNAseq analysis in the cerebral cortex and midbrain/diencephalon/hippocampus areas of Ids-ko mice and wild-type controls at 9 months of age.…”