Get 20M+ Full-Text Papers For Less Than $1.50/day. Start a 14-Day Trial for You or Your Team.

Learn More →

Increase in Glycosaminoglycan Synthesis in Familial Hypercholesterolemic Fibroblasts

Increase in Glycosaminoglycan Synthesis in Familial Hypercholesterolemic Fibroblasts The enzymes of biosynthesis are usually bound to membranes and require an undisturbed lipid environment for regulated activity. In familial hypercholesterolemla, this llpid environment is disturbed and there is a low cholesterol ester level in the cellular membranes that results from impaired processing of low density llpoproteln (LDL). Thus, altered activities of various synthesizing enzymes could be expected. In this study, we found that microsomal glucuronyltransferase activity was 3.4-fold higher in homozygous familial hypercholesterolemic skin fibroblasts (n = 5) than in normal fibroblasts (n = 8). The secretion rates of glycosamlnoglycans in familial hypercholesterolemia were also higher and the incorporation of radiolabeled precursor into glycosaminoglycans was enhanced. In addition to the differences in total synthesis and secretion, we found an altered pattern of individual glycosaminoglycan species with respect to the carbohydrate backbone and a different degree of sulfation in familial hypercholesterolemla. Heparan sulfate with a high degree of sulfation was secreted at a particularly high rate by hypercholesterolemic fibroblasts. Although the affinity of this glycosaminoglycan for LDL Is lower than that of dermatan sulfate, it might cause formation of enhanced LDL-glycosaminoglycan complex and induce the xanthomas and atheromas of familial hypercholesterolemia. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Arteriosclerosis Wolters Kluwer Health

Increase in Glycosaminoglycan Synthesis in Familial Hypercholesterolemic Fibroblasts

Wieczorek, Andreas J.; Zollner, Nepomuk
Arteriosclerosis , Volume 5 (5) – Sep 1, 1985
Download PDF
6 pages

Loading next page...
 
/lp/wolters-kluwer-health/increase-in-glycosaminoglycan-synthesis-in-familial-nFv5U0cf7Z

References

References for this paper are not available at this time. We will be adding them shortly, thank you for your patience.

Copyright
© 1985 by American Heart Association, Inc.
ISSN
0276-5047

Abstract

The enzymes of biosynthesis are usually bound to membranes and require an undisturbed lipid environment for regulated activity. In familial hypercholesterolemla, this llpid environment is disturbed and there is a low cholesterol ester level in the cellular membranes that results from impaired processing of low density llpoproteln (LDL). Thus, altered activities of various synthesizing enzymes could be expected. In this study, we found that microsomal glucuronyltransferase activity was 3.4-fold higher in homozygous familial hypercholesterolemic skin fibroblasts (n = 5) than in normal fibroblasts (n = 8). The secretion rates of glycosamlnoglycans in familial hypercholesterolemia were also higher and the incorporation of radiolabeled precursor into glycosaminoglycans was enhanced. In addition to the differences in total synthesis and secretion, we found an altered pattern of individual glycosaminoglycan species with respect to the carbohydrate backbone and a different degree of sulfation in familial hypercholesterolemla. Heparan sulfate with a high degree of sulfation was secreted at a particularly high rate by hypercholesterolemic fibroblasts. Although the affinity of this glycosaminoglycan for LDL Is lower than that of dermatan sulfate, it might cause formation of enhanced LDL-glycosaminoglycan complex and induce the xanthomas and atheromas of familial hypercholesterolemia.

Journal

Arteriosclerosis – Wolters Kluwer Health

Published: Sep 1, 1985

There are no references for this article.