Can I read Proteoglycan synthesis in normal and Lowe syndrome fibroblasts. on EtoBox?

Proteoglycan synthesis in normal and Lowe syndrome fibroblasts. by G.S. Harper; V.C. Hascall; M. Yanagishita; W.A. Gahl is a Biochemistry, Genetics and Molecular Biology article available to read on EtoBox.

What is Proteoglycan synthesis in normal and Lowe syndrome fibroblasts. about?

Lowe (oculocerebrorenal) syndrome (LS) is an X-linked disorder characterized by congenital cataracts, generalized hypotonia, mental retardation, and renal Fanconi syndrome. The basic defect remains unknown, but the possibility that fibroblasts express reduced sulfation of glycosaminoglycans has been studied in several laboratories. A mechanism involving overproduction of an enzyme (nucleotide pyrophosphatase) active against adenosine 3'-phosphate, 5'-phosphosulfate (PAPS) has been postulated. Decreased synthesis of normally sulfated glycosaminoglycans was also reported. We measured the synthesis of proteoglycans and glycosaminoglycans by incorporation of [3H]glucosamine and Na2(35)SO4 into cultured fibroblasts from four LS patients and related it directly to the synthesis in six normal fibroblast cultures. We found that the rate of synthesis varied greatly among the normal cultures (cv, 30%), but not significantly between LS and the normal. The LS fibroblasts' ability to sulfate glycosaminoglycans was assayed as the amount of 3H-glycosaminoglycan eluting at low ionic strength on anion exchange chromatography, the amount of non-sulfated disaccharide present in chondroitinase digests

Who reads Proteoglycan synthesis in normal and Lowe syndrome fibroblasts.?

Author

G.S. Harper; V.C. Hascall; M. Yanagishita; W.A. Gahl

Publisher

Elsevier BV

Published

1987

Language

EN

Field

Biochemistry, Genetics and Molecular Biology (Life Sciences)