Document Detail

Defective processing of keratan sulfate in macular corneal dystrophy.
MedLine Citation:
PMID:  6238957     Owner:  NLM     Status:  MEDLINE    
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.
K Nakazawa; J R Hassell; V C Hascall; L S Lohmander; D A Newsome; J Krachmer
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  The Journal of biological chemistry     Volume:  259     ISSN:  0021-9258     ISO Abbreviation:  J. Biol. Chem.     Publication Date:  1984 Nov 
Date Detail:
Created Date:  1984-12-27     Completed Date:  1984-12-27     Revised Date:  2003-11-14    
Medline Journal Info:
Nlm Unique ID:  2985121R     Medline TA:  J Biol Chem     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  13751-7     Citation Subset:  IM    
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MeSH Terms
Chromatography, DEAE-Cellulose
Chromatography, Gel
Chromatography, Thin Layer
Cornea / analysis
Glucosamine / metabolism
Glycosaminoglycans / metabolism*
Keratan Sulfate / metabolism*
Macular Degeneration / metabolism*
Mannose / metabolism
Papain / metabolism
Reg. No./Substance:
0/Glycosaminoglycans; 31103-86-3/Mannose; 3416-24-8/Glucosamine; 9056-36-4/Keratan Sulfate; EC

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