Science University Research Symposium (SURS)
Observations of Mitochondrial Morphology in San Fillipo Syndrome
Publication Date
Fall 11-24-2025
College
College of Sciences & Mathematics
Department
Biology, Department of
SURS Faculty Advisor
Felicity Sterling
Presentation Type
Poster Presentation
Abstract
Sanfilippo Syndrome or mucopolysaccharidosis type III (MPS III) is an autosomal recessive lysosomal storage condition that ultimately leads to neurodegeneration due to a pathological accumulation of heparan sulfate (HS) in the lysosome. This leads to lysosomal dysfunction. One core lysosomal function is mitophagy. Impaired mitophagy has been shown to lead to apoptotic cell death, and it hypothesized to be the cause of neurodegeneration in other lysosomal storage disorders. Therefore, we investigated if mitophagy and mitochondrial activity were impaired in Sanfillipo in a manner similar to other lysosomal storage disorders. Mitochondrial movement and flux were severely impaired in Sanfillipo syndrome in comparison to wild type controls. This suggests that mitochondrial activity may be a shared therapeutic target in lysosomal storage disorders.
Recommended Citation
Sterling, Felicity; Armbrecht, Molly; and Walker, Olivia, "Observations of Mitochondrial Morphology in San Fillipo Syndrome" (2025). Science University Research Symposium (SURS). 318.
https://repository.belmont.edu/surs/318
