Defects in the medial entorhinal cortex and dentate gyrus in the mouse model of Sanfilippo syndrome type B.
Sanfilippo syndrome type B (MPS IIIB) is characterized by profound mental retardation in childhood, dementia and death in late adolescence; it is caused by deficiency of α-N-acetylglucosaminidase and resulting lysosomal storage of heparan sulfate. A mouse model, generated by homologous recombination...
Main Authors: | , , |
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Format: | Article |
Language: | English |
Published: |
Public Library of Science (PLoS)
2011-01-01
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Series: | PLoS ONE |
Online Access: | http://europepmc.org/articles/PMC3212581?pdf=render |