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Article

Disease pathology signatures in a mouse model of Mucopolysaccharidosis type IIIB

4 Oct 2023

Abstract excerpt

Mucopolysaccharidosis type IIIB (MPS IIIB) is a rare and devastating childhood-onset lysosomal storage disease caused by complete loss of function of the lysosomal hydrolase α-N-acetylglucosaminidase. The lack of functional enzyme in MPS IIIB patients leads to the progressive accumulation of heparan sulfate throughout the body and triggers a cascade of neuroinflammatory and other biochemical processes ultimately...

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PMID: 37794029
DOI: 10.1038/s41598-023-42431-4

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