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Central nervous system pathology in preclinical MPS IIIB dogs reveals progressive changes in clinically relevant brain regions
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 evaluate novel...
Autores principales: | Egeland, Martin T., Tarczyluk-Wells, Marta M., Asmar, Melissa M., Adintori, Evan G., Lawrence, Roger, Snella, Elizabeth M., Jens, Jackie K., Crawford, Brett E., Wait, Jill C. M., McCullagh, Emma, Pinkstaff, Jason, Cooper, Jonathan D., Ellinwood, N. Matthew |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Nature Publishing Group UK
2020
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7684310/ https://www.ncbi.nlm.nih.gov/pubmed/33230178 http://dx.doi.org/10.1038/s41598-020-77032-y |
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