Serum sulfotransferase levels in patients with macular corneal dystrophy type I.

Abstract

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.

• Authors: J R Hassell, G K Klintworth
• Journal: Archives of ophthalmology (Chicago, Ill. : 1960) (Arch Ophthalmol) Vol. 115 Issue 11 Pg. 1419-21 (Nov 1997) ISSN: 0003-9950 [Print] United States
• PMID: 9366673 (Publication Type: Journal Article, Research Support, U.S. Gov't, P.H.S.)

Chemical References

• Sulfotransferases
• carbohydrate sulfotransferases
• chondroitin 4-sulfotransferase

Topics

• Cornea (enzymology)
• Corneal Dystrophies, Hereditary (enzymology)
• Female
• Humans
• Male
• Sulfotransferases (blood)