Article
Development of sensory, motor and behavioral deficits in the murine model of Sanfilippo syndrome type B.
PloS one - 22 Aug 2007
Heldermon Coy D, Hennig Anne K, Ohlemiller Kevin K, Ogilvie Judith M, Herzog Erik D, Breidenbach Annalisa, Vogler Carole, Wozniak David F, Sands Mark S
Abstract excerpt
BACKGROUND: Mucopolysaccharidosis (MPS) IIIB (Sanfilippo Syndrome type B) is caused by a deficiency in the lysosomal enzyme N-acetyl-glucosaminidase (Naglu). Children with MPS IIIB develop disturbances of sleep, activity levels, coordination, vision, hearing, and mental functioning culminating in early death. The murine model of MPS IIIB demonstrates lysosomal distention in multiple tissues, a shortened life...
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