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Progression of multiple behavioral deficits with various ages of onset in a murine model of Hurler syndrome Pan D; Sciascia A 2nd; Vorhees CV; Williams MTBrain Res 2008[Jan]; 1188 (ä): 241-53Mucopolysaccharidosis type I (MPS I) is one of the most common lysosomal storage diseases with progressive neurological dysfunction. To characterize the chronological behavioral profiles and identify the onset of functional deficits in a MPS I mouse model (IDUA(-/-)), we evaluated anxiety, locomotor behavior, startle, spatial learning and memory with mice at 2, 4, 6 and 8 months of age. In automated open-field test, IDUA(-/-) mice showed hypoactivity as early as 2 months of age and altered anxiety starting from 6 months of age during the initial exploratory phase, even though normal habituation was observed at all ages. In the marble-burying task, the anxiety-like compulsive behavior was normal in IDUA(-/-) mice at almost all tested ages, but significantly reduced in 8-month old male IDUA(-/-) mice which coincided with the rapid death of IDUA(-/-) males starting from 7 months of age. In the Morris water maze, IDUA(-/-) mice exhibited impaired proficient learning only at 4 months of age during the acquisition phase. Spatial memory deficits were observed in IDUA(-/-) mice during both 1 and 7 days probe trials at 4 and 8 months of age. The IDUA(-/-) mice performed normally in a novel object recognition task at younger ages until 8 months old when reduced visual cognitive memory retention was noted in the IDUA(-/-) mice. In addition, 8-month-old IDUA(-/-) mice failed to habituate to repeated open-field exposure, suggesting deficits in non-aversive and non-associative memory. In acoustic startle assessment, significantly more non-responders were found in IDUA(-/-) mice, but normal performance was seen in those that did show a response. These results presented a temporal evaluation of phenotypic behavioral dysfunctions in IDUA(-/-) mice from adolescence to maturity, indicating the impairments, with different ages of onset, in locomotor and anxiety-like compulsive behaviors, spatial learning and memory, visual recognition and short-term non-associative memory retention. This study would also provide guidelines for the experimental designs of behavioral evaluation on innovative therapies for the treatment of MPS type I.|Age of Onset[MESH]|Animals[MESH]|Anxiety Disorders/genetics/metabolism/physiopathology[MESH]|Behavior, Animal/physiology[MESH]|Brain/metabolism/*physiopathology[MESH]|Disease Models, Animal[MESH]|Disease Progression[MESH]|Female[MESH]|Hyperkinesis/genetics/metabolism/physiopathology[MESH]|Iduronidase/*genetics[MESH]|Learning Disabilities/genetics/metabolism/physiopathology[MESH]|Male[MESH]|Memory Disorders/genetics/metabolism/physiopathology[MESH]|Mental Disorders/genetics/metabolism/*physiopathology[MESH]|Mice[MESH]|Mice, Inbred C57BL[MESH]|Mice, Knockout[MESH]|Mucopolysaccharidosis I/genetics/metabolism/*physiopathology[MESH]|Neurocognitive Disorders/genetics/metabolism/*physiopathology[MESH]|Phenotype[MESH]|Reflex, Startle/genetics[MESH] |
