GAG affected in Sanfilippo syndromes is
Correct Answer: Heparan sulfate
Description: Disease name Abbretion Enzyme Defective GaG(s) affected Symptoms Sanfilippo syndrome A MPS IIIA Heparan sulfate-N-sulfatase Heparan sulfate Delay in development, motor dysfunction Sanfilippo syndrome B MPS IIIB a-N-Acetylglucosaminidase Heparan sulfate As MPS IIIA Sanfilippo syndrome C MPS IIIC a-Glucosaminide N-acetyltransferase Heparan sulfate As MPS IIIA Sanfilippo syndrome D MPS IIID N-Acetylglucosamine 6-sulfatase Heparan sulfate As MPS IIIA Reference: Harper; 30th edition; Page no:639; Table: 50-8
Category:
Biochemistry
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