Central nervous system pathology in preclinical MPS IIIB dogs reveals progressive changes in clinically relevant brain regions
Abstract Mucopolysaccharidosis type IIIB (MPS IIIB; Sanfilippo syndrome B) is an autosomal recessive lysosomal storage disorder caused by the deficiency of alpha-N-acetylglucosaminidase activity, leading to increased levels of nondegraded heparan sulfate (HS). A mouse model has been useful to evalua...
Main Authors: | , , , , , , , , , , , , |
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Format: | Article |
Language: | English |
Published: |
Nature Publishing Group
2020-11-01
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Series: | Scientific Reports |
Online Access: | https://doi.org/10.1038/s41598-020-77032-y |