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
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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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