Article
Zebrafish models of Mucopolysaccharidosis IIIA, IIIB, and IIIC show hyperactivity, neuroinflammation and changes in oligodendrocyte cell state
2023-08-03
Abstract excerpt
Sanfilippo syndrome, also known as mucopolysaccharidosis type III (MPS III), is a group of rare inherited lysosomal storage disorders which causes childhood dementia. Subtypes of MPS III are caused by deficiencies in one of four enzymes required for degradation of the glycosaminoglycan heparan sulfate (HS). An inability to degrade HS leads to progressive neurodegeneration and death, often in the second or third de...
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Identifiers and source
- Literature Corpus work
- b07b3f8a-d01b-5f8d-a937-5cfd54c9a371
- DOI
- 10.1101/2023.08.02.550904
