defects in the medial entorhinal cortex and dentate gyrus in the mouse model of sanfilippo syndrome type b缺陷在内侧内嗅皮层和齿状回礼宾部主管综合征小鼠模型的b型.pdfVIP

Defects in the Medial Entorhinal Cortex and Dentate Gyrus in the Mouse Model of Sanfilippo Syndrome Type B Kazuhiro Ohmi, Hui-Zhi Zhao, Elizabeth F. Neufeld* Department of Biological Chemistry, David Geffen School of Medicine, University of California Los Angeles, Los Angeles, California, United States of America Abstract 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 a-N-acetylglucosaminidase and resulting lysosomal storage of heparan sulfate.