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J. Biol. Chem., Vol. 259, Issue 22, 13751-13757, Nov, 1984
K Nakazawa, JR Hassell, VC Hascall, LS Lohmander, DA Newsome and J Krachmer
Macular corneal dystrophy is a human genetic disorder characterized by
corneal opacities that arise, in part, from a failure to synthesize mature
keratan sulfate proteoglycans. The macromolecules in macular corneas and in
keratoconus corneas, an abnormality not involving proteoglycans, were
biosynthetically labeled with [3H]mannose and [14C]glucosamine in organ
culture, and the keratan sulfate proteoglycans were immunoprecipitated with
antibodies against the protein core of monkey keratan sulfate proteoglycan.
The chondroitin sulfate proteoglycans, which did not react with the
antibody, were oversulfated in corneas from patients with macular corneal
dystrophy. Characterization of the immunoprecipitates showed that macular
corneas did not make keratan sulfate proteoglycan but did synthesize an
immunoreactive glycoprotein in nearly equal amounts as keratan sulfate
proteoglycan was synthesized by the keratoconus cornea. The
oligosaccharides on the immunoprecipitated macular glycoprotein appeared to
be normal. However, the macromolecules contained an unsulfated
glycoconjugate that was nearly as large as the normal keratan sulfate
chains isolated from the keratoconus keratan sulfate- proteoglycan and
contained the same relative proportions of labeled glucosamine, mannose,
and fucose. This glycoconjugate was resistant to digestion with keratanase.
These observations indicate that macular corneal dystrophy is caused by an
error in the synthesis of keratan sulfate, possibly involving the specific
sulfotransferases involved in sulfation of the lactosaminoglycan backbone
of the chains.
Defective processing of keratan sulfate in macular corneal dystrophy
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