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...
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Auteurs principaux: | , , , , , , , , , , , , |
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Format: | article |
Langue: | EN |
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Nature Portfolio
2020
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Accès en ligne: | https://doaj.org/article/5500192f556445fb9991fe7f6927c244 |
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