[Various characteristics of the structure and synthesis of procollagens produced by cultured skin fibroblasts from patients with Danlos-Ehlers syndrome type I].
The electrophoretic mobilities of the collagen and procollagen type I and III chains synthesized by the fibroblasts isolated from patients with type I Ehlers-Danlos syndrome as well as a set of peptides obtained by splitting of pro alpha 1(I) and pro alpha 2(I) type I procollagens by cyanbromide are not different from the normal ones. The fact demonstrates the absence of long insertions or deletions, or the sufficient defects in intracellular chain modifications. The changes were also nor registered for the ratio of type I and III collagens from the digested by pepsin preparations of protein accumulating in the culture media of the cultured skin fibroblasts from patients. The studied strains of cultured fibroblasts from patients suffering the Ehlers-Danlos syndrome have the trend to increased accumulation of partially processed chains of proc alpha 1(I) and proc alpha 2(I) type I procollagen and to the increased ratio of pro alpha 1(I) to pro alpha 2(I).
D000595:Amino Acid Sequence; D002478:Cells, Cultured; D004535:Ehlers-Danlos Syndrome / Q000235:genetics / Q000378:metabolism*; D004591:Electrophoresis, Polyacrylamide Gel; D005347:Fibroblasts / Q000378:metabolism; D006801:Humans; D006868:Hydrolysis; D010452:Peptide Biosynthesis; D010455:Peptides / Q000235:genetics; D011347:Procollagen / Q000096:biosynthesis* / Q000235:genetics; D012867:Skin / Q000378:metabolism
D010455:Peptides; D011347:Procollagen
Nekotorye kharakteristiki struktury i sinteza prokollagenov, produtsiruemykh kul'turami fibroblastov kozhi bol'nykh s sintromom Elersa-Danlo I tipa.