Science University Research Symposium (SURS)
Publication Date
Fall 2024
College
Sciences and Mathematics, College of
Department
Biology, Department of
SURS Faculty Advisor
Dr. Felicity Sterling
Presentation Type
Poster Presentation
Abstract
Hurler Syndrome is a rare pediatric neurodegenerative disorder, in which patients experience a multitude of symptoms starting around age 3. These symptoms include skeletal abnormalities, cognitive impairment, stunted growth, heart disease, and many others. The life expectancy of a child diagnosed with Hurler Disease is around 10 to 20 years. Currently, there is no cure for Hurler Syndrome and most treatments are targeted at relieving symptoms. Hurler Syndrome is an inherited lysosomal storage disorder defined by an accumulation of mucopolysaccharides, dermatan sulfate (DS) and heparan sulfate (HS) in tissues. Hurler Syndrome is caused by a mutation in the IDUA gene located on chromosome 4. This mutation leads to the absence of the enzyme alpha-L-iduronidase, which is responsible for breaking down mucopolysaccharides. Previous research conducted on other lysosomal disorders by Sterling Lab revealed that lysosome size increased when cells were drugged with a decrease in lysosomal tubulation in diseased cells.
In this study we treated COS7 cells with ML-SI1 to observe lysosome and mitochondrial activity to model Hurler Disease. It was observed that lysosomal size increased when cells were in a diseased state, treated with 10 uM of ML-SI1. When examining mitochondrial size, it was observed that cells treated with ML-SI1 had a higher percentage of unhealthy mitochondria.
Research Question: How does ML-SI1 impact lysosomal and mitochondrial activity in COS7 cells?
Hypothesis: If ML-SI1 can effectively target motility, there should be a decrease in lysosome and mitochondrial activity.
Prediction: We expect to see an increase in lysosome size and a decrease in healthy appearing mitochondria when COS7 cells are treated with higher doses of ML-SI1.
Recommended Citation
Ryan, Ashlyn and Sebastian, Maria, "Lysosomal Storage Disorder: The Mechanism of Hurler Syndrome" (2024). Science University Research Symposium (SURS). 208.
https://repository.belmont.edu/surs/208
