QUANTITATIVE AND ELECTROPHORETIC ANALYSIS OF COLLAGEN IN CULTURED FIBROBLASTS OF TWO PATIENTS WITH A MILD TYPE I OF OSTEOGENESIS IMPERFECTA DURING TREATMENT WITH BISPHOSPHONATES
Kocova M1,*, Galicka A2, Woczyñski S3, Sukarova-Angelovska E1, Stefanovska I4
*Corresponding Author: Professor Dr. Mirjana Kocova, Pediatric Clinic, Faculty of Medicine, Vodnjanska 17, 1000 Skopje, Republic of Macedonia; Tel: +389-2-3111-713; Fax: +389-2-3176-167; E-mail: ozonunit@unet. com.mk
page: 13

Abstract

Electrophoretic analysis of procollagen synthesized and secreted by cultured skin fibroblasts from two patients with a mild type of osteogenesis imperfecta (OI) treated with bisphosphonates is presented. Analysis was performed 20 months and 9 months, respectively, after the initiation of the treatment. The ratio of type III to type I procollagen markedly increased above the control level, suggesting decreased synthesis of type I procollagen and normal level of type III procollagen, a pattern characteristic for OI type I. Collagen biosynthesis (measured by L-[5-3H]proline incorporation into proteins susceptible to bacterial colla­genase) was comparable to that of the control in both pa­tients. It can be caused by the increased level of type III collagen, seen on the gel where the collagens of types I and III were separated in non reducing conditions. There was no significant difference in migration of collagen chains, indicating absence of the abnormal collagen. How­ever, secretion of collagen into medium was 19 and 28% less, respectively, than in the control. An increased level of type III collagen with normal structure may be a com­pensatory response to the low production of type I collagen in the mild type I of OI as a result of therapy with biphos­phonates.

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1 Pediatric Clinic, Faculty of Medicine, 1000 Skopje, Re­public of Macedonia

2 Department of Medical Chemistry, Medical University of Bialystok, Bialystok, Poland

3 Department of Gynaecological Endocrinology, Medical University of Bialystok, Bialystok, Poland

4 Orthopedic Clinic, Faculty of Medicine, 1000 Skopje, Republic of Macedonia




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