Serum sulfotransferase levels in patients with macular corneal dystrophy type I
J. R. Hassell and G. K. Klintworth
Department of Ophthalmology, University of Pittsburgh School of Medicine, Pa., USA.
OBJECTIVE: To measure the levels of sulfotransferase activity for keratan
sulfate and chondroitin sulfate in serum of patients with macular corneal
dystrophy type I, an inherited disorder that is characterized by the
absence of sulfate esters on keratan sulfate in the corneal stroma.
METHODS: The amount of sulfur-35 transferred from 3'-phosphoadenosine
5'-phosphosulfate to partially sulfated keratan sulfate and partially
sulfated chondroitin sulfate by the sulfotransferase present in serum from
patients with macular corneal dystrophy and age-matched controls was
determined under conditions where only the added enzyme was rate limiting.
RESULTS: Serum from patients with macular corneal dystrophy type I has the
same level of sulfotransferase activity for keratan sulfate and chondroitin
sulfate as found in age-matched controls. CONCLUSIONS: Patients with
macular corneal dystrophy type I have sulfotransferase activity for
sulfating at least 1 of the 2 sugars in keratan sulfate. It is proposed
that the sulfotransferase for N-acetylglucosamine may be deficient.
