Mucopolysaccharidosis is a lysosomal storage disease, in which the body cannot degrade the glycosaminoglycans. These compounds are accumulating in the body’s cells. We can divide this disorder to 11 main groups on the basis of deficient or absent lysosomal enzymes. We know that in 7 groups an increased amount of glycosaminoglycans can be detected in the urine.
My objective was to measure the amount of glycosaminoglycans in the urine.
I used Simadzu UV-1800 spectrophotometer for measurements and the data evaluation was done by Simadzu UV Probe software.
During my work I studied the stability and storage life of the reaction solutions, samples and the measuring complex. I characterized the static characteristic of the calibration curve. Hereby I get more information about the analytical parameters and applications of this method. Furthermore, I tested whether some other urine components could cause interference during the measurement. I have examined proteins, globulins and creatinine. I investigated the usability of the creatinine/GAG rate and I tried to get around the interference of proteins.
Today I have led in a precise and useable routine laboratory diagnostic method to the MPS screening and therapy monitoring.