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Literature DB >> 7206599

Osteogenesis imperfecta: biochemical and clinical evaluation of 13 cases.

T Krieg, E Kirsch, K Matzen, P K Müller.

Abstract

Skin fibroblasts were cultured from 13 patients with Osteogenesis imperfecta and collagen biosynthesis was investigated in vitro. In those patients characterised by only mild manifestations of the disease, the ratio of collagen types I and III was disturbed. By contrast, fibroblasts obtained from patients with Osteogenesis imperfecta of a more severe type synthesised collagen types I/III in a normal ratio.

Entities: Disease Species

Mesh：

Substances：
Collagen

Year: 1981 PMID： 7206599 DOI： 10.1007/bf01477288

Source DB: PubMed Journal: Klin Wochenschr ISSN： 0023-2173

13 in total

1. Biochemical characteristics and biological significance of the genetically-distinct collagens.

Authors: E J Miller
Journal: Mol Cell Biochem Date: 1976-12-10 Impact factor: 3.396

2. The marfan's syndrome. In vitro study of collagen metabolism in tissue specimens of the aorta.

Authors: T Krieg; P K Müller
Journal: Exp Cell Biol Date: 1977

3. Synthesis and degradation of collagen by skin fibroblasts from controls and from patients with osteogenesis imperfecta.

Authors: B U Steinmann; G R Martin; B I Baum; R G Crystal
Journal: FEBS Lett Date: 1979-05-15 Impact factor: 4.124

4. Altered relation of two collagen types in osteogenesis imperfecta.

Authors: B Sykes; M J Francis; R Smith
Journal: N Engl J Med Date: 1977-05-26 Impact factor: 91.245

5. Synthesis of collagen by human fibroblasts and their SV40 transformants.

Authors: T Krieg; M Aumailley; W Dessau; M Wiestner; P Müller
Journal: Exp Cell Res Date: 1980-01 Impact factor: 3.905

6. Isolation and characterization of the peptides derived from soluble human and baboon skin collagen after cyanogen bromide cleavage.

Authors: E H Epstein; R D Scott; E J Miller; K A Piez
Journal: J Biol Chem Date: 1971-03-25 Impact factor: 5.157

Osteogenesis imperfecta: biochemical and clinical evaluation of 13 cases.

1. Biochemical characteristics and biological significance of the genetically-distinct collagens.

2. The marfan's syndrome. In vitro study of collagen metabolism in tissue specimens of the aorta.

3. Synthesis and degradation of collagen by skin fibroblasts from controls and from patients with osteogenesis imperfecta.

4. Altered relation of two collagen types in osteogenesis imperfecta.

5. Synthesis of collagen by human fibroblasts and their SV40 transformants.

6. Isolation and characterization of the peptides derived from soluble human and baboon skin collagen after cyanogen bromide cleavage.

7. Osteogenesis imperfecta congenita: evidence for a generalized molecular disorder of collagen.

8. Genetic heterogeneity in osteogenesis imperfecta.

9. Abnormal collagen metabolism in cultured cells in osteogenesis imperfecta.

10. Patients with Ehlers-Danlos syndrome type IV lack type III collagen.

1. Type I osteogenesis imperfecta: a nonfunctional allele for pro alpha 1 (I) chains of type I procollagen.

2. Influence of cell density on collagen biosynthesis in fibroblast cultures.

3. Altered glycosaminoglycan production in cultured osteogenesis-imperfecta skin fibroblasts.

4. Heterogeneity of osteogenesis imperfecta type I.

5. Abnormal alpha 2-chain in type I collagen from a patient with a form of osteogenesis imperfecta.

6. Vertebral bone mineral content in osteogenesis imperfecta.