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

Published

Journal Article

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.

Full Text

Cited Authors

  • Hassell, JR; Klintworth, GK

Published Date

  • November 1997

Published In

Volume / Issue

  • 115 / 11

Start / End Page

  • 1419 - 1421

PubMed ID

  • 9366673

Pubmed Central ID

  • 9366673

International Standard Serial Number (ISSN)

  • 0003-9950

Digital Object Identifier (DOI)

  • 10.1001/archopht.1997.01100160589011

Language

  • eng

Conference Location

  • United States