Abstract
PG, with collagen and elastin, are major constituents of connective tissue. No consistant genetic biochemical abnormality has been found in 01. Skin fibroblasts from patients with 01-1, both with (N=3), and without (N=3), detinogenesis imperfecta (DI), were labeled with 35S-SO4 for 48 hours. Medium (M) and cell layer (CL) macromolecular material (35S-PG) was fractionated from 35S-SO4, and expressed as counts per minute (CPM)/mgm CL protein (P) or CPM/103 cells. No quantitative differences were seen in the two categories of 01-1 fibroblasts. Autoradiography of 35S-PG (M and CL) from dried large-pore polyacrylamide-agarose (LPPA) electrophoretic gels showed no major qualitative differences between the pattern in cells from 01-1, with or without D1. Qualitative and quantitative differences seen between the two 01 groups and controls may be related to age. Cartilage (CA) from patients with 01-11 (N=3) and controls (N=2) was maintained in organ culture. Approximately 100 mgm wet weight CA was labeled for 48 hrs with 35S-SO4. 35S-PG in M and in CA extracts (CE, extraction in 4M GnCl of the labeled CA) revealed no major quantitative differences in CPM/mgm P or CPM/103 cells, although individual values were variable in the M. However, autoradiography in LPPA gels revealed an extra cathodal 35S-PG band from CE occurring in the 01-11 cartilage, not present in the control samples. Identification by enzymatic degredation is being pursued.
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Ward, J., Levin, L. & Summitt, R. 771 SKIN FIBROBLAST AND CARTILAGE PROTEOGLYCAMS (PG) IN OSTEOGENESIS IMPERFECTA (01) TYPES I AND II. Pediatr Res 15 (Suppl 4), 571 (1981). https://doi.org/10.1203/00006450-198104001-00795
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DOI: https://doi.org/10.1203/00006450-198104001-00795