Synthesis of structurally unstable type III procollagen in patients with cerebral artery aneurysm.
The biosynthesis of collagen was studied in skin fibroblast cultures established from 11 patients with cerebral artery aneurysms. Six patients had familial subarachnoid hemorrhage (SAH), while five patients were considered as sporadic cases. The structural stability of the triple-helical medium procollagen was studied by measuring the thermal denaturation temperature (Tm) of type I and type III procollagen molecules. Structural instability of type III procollagen was demonstrated in two patients with familial SAH. The Tm of type III procollagen was 39.0 degrees C and 39.5 degrees C in two of the cell lines, while the control value was 40.3 degrees C. The stability of type I procollagen did not differ from that of the controls, and the main features of the biosynthesis of collagen were similar in the aneurysm patient cell lines and in the controls. The results suggest that a structural defect of type III procollagen may serve as an etiological factor in the formation of cerebral artery aneurysms.
Majamaa
K
K
Department of Neurology, University of Oulu, Finland.
Savolainen
E R
ER
Myllylä
V V
VV
D000328:Adult; D002478:Cells, Cultured; D005260:Female; D006801:Humans; D002532:Intracranial Aneurysm / Q000209:etiology / Q000378:metabolism*; D008297:Male; D008875:Middle Aged; D009693:Nucleic Acid Hybridization; D010449:Peptide Mapping; D011347:Procollagen / Q000096:biosynthesis* / Q000737:chemistry; D011487:Protein Conformation; D011499:Protein Processing, Post-Translational; D012333:RNA, Messenger / Q000235:genetics / Q000378:metabolism; D013345:Subarachnoid Hemorrhage / Q000209:etiology / Q000378:metabolism; D013696:Temperature
D011347:Procollagen; D012333:RNA, Messenger