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...

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Detalles Bibliográficos
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
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group UK 2020
Materias:
Article
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

Internet

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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