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Evaluation Of Seizure Threshold As An Early Behavioral Marker Of Disease Progression In The Mouse Model Of Mucopolysaccharidosis Iiia, Pamela Santiago
Evaluation Of Seizure Threshold As An Early Behavioral Marker Of Disease Progression In The Mouse Model Of Mucopolysaccharidosis Iiia, Pamela Santiago
Dissertations, Masters Theses, Capstones, and Culminating Projects
Mucopolysaccharidosis IIIA (MPS IIIA) is a lysosomal storage disease caused by a mutation in the gene that codes for the enzyme heparan sulfamidase. The decreased enzyme activity of heparan sulfamidase results in the accumulation of heparan sulfate (HS). HS accumulation in the brain causes severe central nervous system (CNS) complications, including learning and memory deficits and seizure. MPS IIIA patients have short life expectancies and there currently is no cure for the disease. This thesis work was aimed at identifying an early neurological phenotype in the mouse model of MPS IIIA. The data will aid in the design of an …