Altered synthesis of Bruch's membrane proteoglycans associated with dominant retinitis pigmentosa.
Proteoglycans, extracellular matrix molecules that have been shown to have filtration properties in some tissues, make up a significant proportion of the structural macromolecules of Bruch's membrane. Bruch's membrane may provide a selective filtration barrier between the choriocapillaris and the pigmented epithelium (PE) and outer retina. In this paper, we compare the proteoglycans extracted from metabolically-labeled normal and retinitis pigmentosa (RP) Bruch's membranes. Isolated RP Bruch's membrane proteoglycans were larger than those from normal donor eyes when chromatographed on a column of Sepharose CL-4B. In addition to the increased size, there was also a dramatic increase in the proportion of heparan sulfate proteoglycan being synthesized in RP. Considering the structural and filtration properties of proteoglycans, alterations such as these could affect the functioning of Bruch's membrane and, possibly, the PE and the outer retina.
D002809:Chondroitin Sulfates / Q000378:metabolism; D002829:Choroid / Q000378:metabolism*; D002845:Chromatography; D003871:Dermatan Sulfate / Q000378:metabolism; D005799:Genes, Dominant; D006497:Heparan Sulfate / Q000378:metabolism; D006801:Humans; D008970:Molecular Weight; D011509:Proteoglycans / Q000378:metabolism*; D012016:Reference Values; D012174:Retinitis Pigmentosa / Q000235:genetics / Q000378:metabolism*
D002809:Chondroitin Sulfates; D003871:Dermatan Sulfate; D006497:Heparan Sulfate; D011509:Proteoglycans