PathwayStep1099 PathwayStep1100 PathwayStep1107 PathwayStep1105 PathwayStep1106 PathwayStep1103 PathwayStep1104 PathwayStep1101 PathwayStep1102 CS/DS degradation Authored: Jassal, B, 2011-12-14 Edited: Jassal, B, 2011-12-14 GENE ONTOLOGYGO:0030207 Lysosomal degradation of glycoproteins is part of the cellular homeostasis of glycosylation (Winchester 2005). The steps outlined below describe the degradation of chondroitin sulfate and dermatan sulfate. Complete degradation of glycoproteins is required to avoid build up of glycosaminoglycan fragments which can cause lysosomal storage diseases. Complete degradation steps are not shown as they are repetitions of the main ones described here. The proteolysis of the core protein of the glycoprotein is not shown here. Pubmed15647514 Reactome Database ID Release 432024101 Reactome, http://www.reactome.org ReactomeREACT_120888 Reviewed: D'Eustachio, P, 2012-03-28 DS is cleaved from its proteoglycan CS is cleaved from its proteoglycan DSPG and CSPG translocate to the lysosome for degradation Hyaluronidase 1 (HYAL1) hydrolyses 1-4 linkages between GalNAc and D-glucuronate residues in CS. Arylsulfatase B (ARSB) hydrolyses sulfate groups from GalNAc 4-sulfate units of CS Iduronate 2-sulfatase (IDS) hydrolyses sulfates from L-iduronate 2-sulfate units of dermatan sulfate Arylsulfatase B (ARSB) hydrolyses sulfate groups from GalNAc 4-sulfate units of DS Alpha-L-iduronidase (IDUA) hydrolyses the unsulfated alpha-L-iduronosidic linkage in DS Beta-hexosaminidase A (BHEXA) cleaves the terminal GalNAc from DS