Dermatan sulfate sulfation patterns

Vilela-Silva et ah, 1999, 2002). The body of some species of ascidians also contains peculiar GAGs that show distinct sulfation patterns from mammalian GAGs (Pavao et ah, 1995, 1998) as exemplified with the tunicate dermatan sulfates (DSs) in Fig. 12.2F. 

Pavao, M. S., Mourao, P. A., Mulloy, B., and Tollefsen, D. M. (1995). A unique dermatan sulfate-like glycosaminoglycan from ascidian. Its structure and the effect of its unusual sulfation pattern on anticoagulant activity. J. Biol. Chem. 270,31027-31036. 

Vicente, . P., Zancan, P., Peixoto, L. L., Alves-Sa, R., Araujo, F. S., Mourao, P. A., and Pavao, M. S. (2001). Unbalanced effects of dermatan sulfates with different sulfation patterns on coagulation, thrombosis and bleeding. Thromb. Haemost. 86,1215-1220. 

Spot patterns should always be assessed relative to the standard mixture. In general, keratan sulfate will migrate with the leading front, while chondroitin-6-sul-fate should be located between fronts 5 and 6, chondroitin-4-sulfate between fronts 4 and 5, heparan sulfate between fronts 3 and 4, hyaluronic acid between fronts 2 and 3, and dermatan sulfate between fronts 1 and 2. 

Typical patterns of GAG excretion can be found in the urine of MPS patients [37]. For MPS I and II an increased excretion of dermatan and heparan sulfate is found, with the first being especially pronounced on TLC. In case of MPS III, heparan sulfate will be increased, while a prominent keratan sulfate band characterizes MPS IV. MPS VI shows slightly increased dermatan sulfate excretion that can also be found for the MPS I Scheie variant. 

The pattern of spots is evaluated in reference to the standard mixture. Dermatan sulfate yields two spots, one of which can overlap with hyaluronic acid. Abnormal presence of dermatan sulfate and heparan sulfate is an indication of MPS I or MPS II, while a prominent heparan sulfate spot points to MPS III. The subtypes of MPS III can only be differentiated by enzyme studies. Elevated excretion of keratan sulfate is indicative of MPS IV. In contrast, high dermatan sulfate excretion is most likely to be associated with MPS VI, while milder cases of MPS I may also show such a pattern (cf. Table 4.1.5) [56]. 

Some disorders may cause an abnormal excretion of GAGs in urine. Among those are various bone diseases, connective tissue diseases, hypothyroidism, urinary dysfunction, and aspartylglucosaminuria. Faint spots for keratan sulfate can also be found in spondyloepiphyseal dysplasia as well as in type II mucohpidoses, where traces of dermatan sulfate may additionally be visible. Furthermore, heparin and tris(hydroxymethyl)aminomethane (Trometamol) can interfere with electrophoretic patterns [56]. To provide a good interpretation of results the clinical features of the patient and the current therapy have to be known. Furthermore, a study of the urin-... 

C.D. Nandini, N. Itoh, K. Sugahara, Novel 70-kDa chondroitin sul-fate/dermatan sulfate hybrid chains with a unique heterogeneous sulfation pattern from shark skin, which exhibit neuritogenic activity and binding activities for growth factors and neurotrophic factors, J. Biol. Chem. 280 (6) (2005) 4058-4069. 

---