Dermatan sulphate

Glycosamanoglucan belongs to the chondroitin sulfates (old name: mucopolysaccharide).

Chondroitin sulphates are divided into 2 forms:

• Chondroitin sulphate A: Consists of glycosidically linked glucuronic acid and N-acetylgalactosamine
• Chondroitin sulphate B (dermatan sulphate): Consists of the glycosidically linked L-iduronic acid and N-acetylgalactosamine (see below chondroitin sulphate).

In Pfaundler-Hurler disease, an autosomal recessive mutation of the α-iduronidase gene (gene locus: 4p16.3), a lysosomal degradation of dermatan sulfate (= chondroitin sulfate B) and heparan sulfate occurs in skin and internal organs.

The very rare musculo-contractival Ehlers-Danlos syndrome (mcEDS) is characterized by autosomal recessive inheritance. Mutations in the genes CHST14 and DSE are responsible for mcEDS. The CHST14 gene codes for dermatansulfotransferase-1.

1. Mizumoto S et al (2015) Mutations in Biosynthetic Enzymes for the Protein Linker Region of Chondroitin/Dermatan/Heparan Sulfate Cause Skeletal and Skin Dysplasias. Biomed Res Int 2015:861752.
2. Vigan M (2014) Allergic contact dermatitis caused by sodium chondroitin sulfate contained in a cosmetic cream. Contact dermatitis 70:383-384.