Activities of N-acetylgalactosamine-6-sulfate sulfatase in liver from two sisters with morquio syndrome.
A 6-sulfated tetrasaccharide obtained by digesting chondroitin-6-sulfate with testicular hyaluronidase was used as a substrate for the determination of N-acetylgalactosamine-6-sulfate sulfatase activity. The activity was not detected in liver obtained from the elder sister with clinically classic Morquio syndrome and 4.7% of the control in liver from the younger sister with the same disease.
D000293:Adolescent; D002810:Chondroitinases and Chondroitin Lyases / Q000378:metabolism*; D002811:Chondroitinsulfatases / Q000378:metabolism*; D005260:Female; D006801:Humans; D008099:Liver / Q000201:enzymology*; D009085:Mucopolysaccharidosis IV / Q000201:enzymology* / Q000235:genetics
D002811:Chondroitinsulfatases; D002810:Chondroitinases and Chondroitin Lyases