Morphologic and immunofluorescent studies of the cytoskeleton of fibroblasts from patients with osteogenesis imperfecta

Date
1984
DOI
Authors
Nieto-Herman, Aidee
Version
OA Version
Citation
Abstract
Morphologic and immunocytoskeletal features were studied in skin fibroblasts cultured from patients with Osteogenesis Imperfecta (OI) Type 1 and from normal human embryonic lung. The cytoskeleton was stained with fluorescein labelled Nitrobenzoxadiazole phallacidin (NBD-ph). Normal skin fibroblasts were generally elongated and stellate shaped with trailing cell processes. The F-Actin stress fibers extended throughout the cell. It was found in significant amount in the cytoplasm in microfilament bundles and in punctate islands. These cells showed regular patterns of cell growth with cells orienting themselves in longitudinal, swirling patterns. In OI cells, actin containing filaments were atypical with a fragmented intracellular network in discontinuous lines, irregular patches, and rosettes. These cells showed irregular patterns of growth, the cells were more rounded and irregularly shaped. There was no orientation along a central axis to give an elongated shape as was seen in the normal fibroblasts. Our results suggest a relationship between the F-actin and the containing components of the cytoskeleton, cell shape, and growth patterns of fibroblasts from patients with O.I.
Description
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Includes photomicrographs.
Thesis (M.Sc.D.)--Boston University, Henry M. Goldman School of Graduate Dentistry, 1984 (Oral biology)
Bibliography : leaves 49-58.
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