Article
Molecular Modeling of N-Acetylglucosamine Binding to the I154R Mutant of NAGLU: Pathogenic Insights into Sanfilippo Syndrome Type B.
International journal of molecular sciences - 15 May 2026
Kannan Priyanka, Priya Nanda Kumar Madhana, Kumar Nanda Kumar Sidharth, Vasudevan Vasundra, Kaviarasan Kuppan, Ramasamy Magesh
Abstract excerpt
Sanfilippo syndrome type B, also known as mucopolysaccharidosis type IIIB (MPS IIIB), is a rare autosomal recessive lysosomal storage disorder caused by mutations in the N-acetyl-α-D-glucosaminidase (NAGLU) gene, which encodes the enzyme α-N-acetylglucosaminidase. This enzyme is essential for degrading heparan sulfate. The deficiency leads to toxic accumulation within cells. To investigate the impact of NAGLU...
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