A DISORDER OF COLLAGEN BIOSYNTHESIS IN PATIENTS WITH CEREBRAL-ARTERY ANEURYSM

Citation
K. Majamaa et Vv. Myllyla, A DISORDER OF COLLAGEN BIOSYNTHESIS IN PATIENTS WITH CEREBRAL-ARTERY ANEURYSM, Biochimica et biophysica acta, 1225(1), 1993, pp. 48-52
Citations number
32
Language
INGLESE
art.tipo
Article
Categorie Soggetti
Biophysics,Biology
ISSN journal
0006-3002
Volume
1225
Issue
1
Year of publication
1993
Pages
48 - 52
Database
ISI
SICI code
0006-3002(1993)1225:1<48:ADOCBI>2.0.ZU;2-7
Abstract
12 patients with subarachnoid hemorrhage due to rupture of a cerebral aneurysm were examined clinically for symptoms and signs of a connecti ve tissue disorder and biochemically for details of the biosynthesis o f collagen. No uniform clinical pattern of any connective tissue disor der was seen in these patients, although selected signs were observed. Skin fibroblast cultures were then established. The rate of procollag en production in two cell lines was reduced by 40% and 50%, respective ly, and the intracellular accumulation of hydroxy[C-14]proline (as a p ercentage of total hydroxy[C-14]proline) was increased by 70% in each relative to eight control cell lines. No difference was found in the d egree of intracellular degradation of procollagen. After pulse-labelli ng, however, the radioactive procollagen was secreted into the medium in 1 h in the control cells, but required at feast 3 h in the two aneu rysm patient cell lines. The results, thus, suggest that delayed secre tion of procollagen rather than increased intracellular degradation le d to the reduction in the rate of procollagen synthesis in these two f ibroblast lines from patients with cerebral artery aneurysm.