IDUA
UniProt:P35475 IDUA
HYAL1
LUCA1
UniProt:Q12794 HYAL1
DSPG and CSPG translocate to the lysosome for degradation
DS is cleaved from its proteoglycan
CS is cleaved from its proteoglycan
Arylsulfatase B (ARSB) hydrolyses sulfate groups from GalNAc 4-sulfate units of DS
Iduronate 2-sulfatase (IDS) hydrolyses sulfates from L-iduronate 2-sulfate units of dermatan sulfate
Beta-hexosaminidase A (BHEXA) cleaves the terminal GalNAc from DS
Alpha-L-iduronidase (IDUA) hydrolyses the unsulfated alpha-L-iduronosidic linkage in DS
Arylsulfatase B (ARSB) hydrolyses sulfate groups from GalNAc 4-sulfate units of CS
Hyaluronidase 1 (HYAL1) hydrolyses 1-4 linkages between GalNAc and D-glucuronate residues in CS.
CS/DS degradation