dermatan sulfate

Dermatan sulfate (formerly called chondroitin sulfate B) is a glycosaminoglycan found predominantly in connective tissue, skin, blood vessels, and heart valves. Structurally, it consists of alternating units of N-acetylgalactosamine (often sulfated) and L-iduronic acid, linked to core proteins to form proteoglycans. It plays roles in wound healing, cell signaling, and interacts with coagulation factors (it can activate heparin cofactor II, contributing to its anticoagulant properties).

Clinically, dermatan sulfate is most relevant in the context of mucopolysaccharidoses (MPS)—a group of lysosomal storage diseases caused by deficiencies in enzymes required for GAG degradation. Two key examples:

- Hurler syndrome (MPS I): deficiency of alpha-L-iduronidase, leading to accumulation of both dermatan sulfate and heparan sulfate. Presents with coarse facial features, corneal clouding, hepatosplenomegaly, skeletal abnormalities, and intellectual disability.

- Hunter syndrome (MPS II): deficiency of iduronate-2-sulfatase, also causing accumulation of dermatan sulfate and heparan sulfate, but without corneal clouding and with X-linked recessive inheritance (unlike Hurler, which is autosomal recessive).

Accumulated dermatan sulfate in these disorders contributes to tissue damage, organomegaly, and skeletal deformities, and can be detected via elevated urinary GAG excretion.

Additionally, dermatan sulfate has been studied for its anticoagulant potential due to its interaction with heparin cofactor II, though it is much less potent than heparin, which primarily works via antithrombin III.