Purchase this article with an account.
Nobuko Hasegawa, Takayoshi Torii, Takuji Kato, Hiroaki Miyajima, Atsushi Furuhata, Kiyoo Nakayasu, Atsushi Kanai, Osami Habuchi; Decreased GlcNAc 6-O-Sulfotransferase Activity in the Cornea with Macular Corneal Dystrophy. Invest. Ophthalmol. Vis. Sci. 2000;41(12):3670-3677.
Download citation file:
© ARVO (1962-2015); The Authors (2016-present)
purpose. Macular corneal dystrophy (MCD) is an autosomal recessive inherited
disorder that is accompanied by corneal opacity. Explants from
MCD-affected corneas have been reported to synthesize low-sulfated KS,
suggesting that sulfate groups attached to KS may play critical roles
in maintaining corneal transparency. To clear the biosynthetic defect
in the MCD cornea, sulfotransferase activities were determined that are
presumably involved in the biosynthesis of KS:
galactose-6-sulfotransferase (Gal6ST) activity and N-acetylglucosamine 6-O-sulfotransferase
methods. Gal6ST and GlcNAc6ST activities, which were contained in the corneal
extracts from corneas affected by MCD and keratoconus and from normal
control corneas, were determined by measuring the transfer of 35SO4 from[ 35S]3′-phosphoadenosine 5′-phosphosulfate into the Gal
residue of partially desulfated KS and the nonreducing terminal GlcNAc
residue of GlcNAcβ1-3Galβ1-4GlcNAc (oligo A), respectively.
results. The level of Gal6ST activity in corneal extracts from eyes with MCD,
which was measured by using partially desulfated KS as an acceptor, was
nearly equal to that in eyes with keratoconus and normal control eyes.
In contrast, GlcNAc6ST activity in the extracts from MCD-affected
corneas, which was measured by using oligo A as an acceptor, was much
lower than in those in corneas with keratoconus and in normal control
conclusions. The decrease in GlcNAc6ST activity in the cornea with MCD may result in
the occurrence of low- or nonsulfated KS and thereby cause corneal
This PDF is available to Subscribers Only