Glycosaminoglycans:mucopolysaccharides Flashcards
Hyaluronate
D-Glucosamine, D-Glucuronate
Chondroitin Sulfate
D-Galactosamine, D-Glucuronate
Dermatan Sulfate
D-Galactosamine, L-iduronate/D-Glucuronate
Keratin sulfate
D-Glucosamine, none (but contains D-Galactose
Heparin Sulfate
D-Glucosamine, D-Glucuronate (major)- L-Iduronate (minor)
Heparin
D-Glucosamine, L-iduronate (major)- D-Glucuronate (minor)
MPS I H
Hurler, deficient in enzyme alpha-L-Iduronate affects dermatan sulfate and heparin sulfate
MPS I S
Scheie, deficient in enzyme alpha-L-Iduronate affects dermatan sulfate and heparin sulfate
MPS H/S
Hurler/Scheie, deficient in enzyme alpha-L-Iduronate affects dermatan sulfate and heparin sulfate
MPS II (severe)
Hunter (severe), deficient in enzyme Iduronate sulfatase affects dermatan sulfate and heparin sulfate
MPS II (mild)
Hunter (mild), deficient in enzyme Iduronate sulfatase affects dermatan sulfate and heparin sulfate