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Article

Progression of multiple behavioral deficits with various ages of onset in a murine model of Hurler syndrome.

Brain research - 10 Jan 2008

Pan Dao, Sciascia Anthony, Vorhees Charles V, Williams Michael T

Abstract excerpt

Mucopolysaccharidosis 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...

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Source

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Identifiers

PMID: 18022143
DOI: 10.1016/j.brainres.2007.10.036
PMCID: PMC2323205

Genes

IDUA

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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