Article
Development of motor deficits in a murine model of mucopolysaccharidosis type IIIA (MPS-IIIA).
Behavioural brain research - 30 Mar 2005
Hemsley Kim M, Hopwood John J
Abstract excerpt
Mucopolysaccharidosis (MPS) type IIIA or Sanfilippo syndrome is a lysosomal storage disorder characterised by progressive neurological pathology. Patients exhibit aggression, disturbed sleep, hyperactivity and mental decline ultimately resulting in inanition and death. Recently, a mouse model of MPS-IIIA was discovered, and both the clinical signs and neuropathological changes mimic the human disease. This...
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