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Entry Name Description Category Pathway Gene
H00493 Heparan sulfate proteoglycan gene defects Defects in heparan sulfate proteoglycans (HSPGs), which present in cartilage, are associated with skeletal growth disorders. Mutations in either HS biosynthetic enzymes or HS proteoglycan core proteins ... Congenital malformation (DDSH/SJS1) HSPG2 [HSA:3339] [KO:K06255]
(SGBS1) GPC3 [HSA:2719] [KO:K08109]
(OMOD1) GPC6 [HSA:10082] [KO:K08112]
(EXT1) EXT1 [HSA:2131] [KO:K02366]
(EXT2) EXT2 [HSA:2132] [KO:K02367]
H02154 Omodysplasia ... craniofacial dysmorphism, and variable developmental delay. It is caused by mutations in glypican 6 (GPC6). An autosomal dominant form (OMOD2) involving only the upper limbs was later recognized as a separate ... Congenital malformation (OMOD1) GPC6 [HSA:10082] [KO:K08112]
(OMOD2) FZD2 [HSA:2535] [KO:K02235]
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