Serum sulfotransferase levels in patients with macular corneal dystrophy type I.
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.
Hassell
J R
JR
Department of Ophthalmology, University of Pittsburgh School of Medicine, Pa., USA.
Klintworth
G K
GK
D003315:Cornea / Q000201:enzymology; D003317:Corneal Dystrophies, Hereditary / Q000201:enzymology*; D005260:Female; D006801:Humans; D008297:Male; D015238:Sulfotransferases / Q000097:blood*; D000097811:Carbohydrate Sulfotransferases
D015238:Sulfotransferases; D000097811:Carbohydrate Sulfotransferases; C019893:chondroitin 4-sulfotransferase