Macular corneal dystrophy: failure to synthesize a mature keratan sulfate proteoglycan.
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منابع مشابه
Macular corneal dystrophy: failure to synthesize a mature keratan sulfate proteoglycan.
Corneal specimens obtained during surgery from patients with macular corneal dystrophy and obtained at autopsy from control eyes were incubated in a medium containing radioactive precursors of glycoproteins and proteoglycans. Biosynthetically radiolabeled material was extracted and characterized by using molecular sieve chromatography and specific enzymes. Cells in control corneas synthesized b...
متن کاملDefective processing of keratan sulfate in macular corneal dystrophy.
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 ...
متن کاملDistribution of proteoglycans antigenically related to corneal keratan sulfate proteoglycan.
Three antibodies reacting with corneal keratan sulfate proteoglycan were used to detect antigenically related molecules in 11 bovine and 13 embryonic chick tissues. Two monoclonal antibodies recognized sulfated epitopes on the keratan sulfate chain and a polyclonal antibody bound antigenic sites on the core protein of corneal keratan sulfate proteoglycan. Competitive immunoassay detected core p...
متن کاملMacrophage receptors for lumican. A corneal keratan sulfate proteoglycan.
PURPOSE Keratan sulfate proteoglycans (KSPGs) of the cornea exhibit a characteristic change in glycosylation resulting from stromal inflammation and scarring. To examine potential roles for these molecules in the pathobiology of the cornea, the authors investigated interaction of inflammatory macrophages with KSPGs in vitro. METHODS Attachment and spreading of mouse peritoneal macrophages wer...
متن کاملImmunoanalysis of keratan sulfate proteoglycan from corneal scars.
Corneal keratan sulfate proteoglycan (KSPG) from scar tissue of experimental penetrating corneal wounds in rabbits was analyzed 2-8 weeks after injury using three previously characterized antibodies. Keratan sulfate (KS) was identified in 2 week scars and normal corneal tissue by indirect immunofluorescence using a monoclonal antibody against sulfated KS epitopes. KSPG was measured in unfractio...
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ژورنال
عنوان ژورنال: Proceedings of the National Academy of Sciences
سال: 1980
ISSN: 0027-8424,1091-6490
DOI: 10.1073/pnas.77.6.3705