Abnormal gene expressions of stroma cells in patients with tuberous sclerosis.
The characteristics of stroma cells in tissues and the cloned cells, which derived from adenomata sebacea in the skin of patients with tuberous sclerosis, were investigated using histochemical, biochemical, flow cytofluorometric, electron microscopic, and immunofluorescent techniques, and the following results were obtained: 1. The stroma cells from adenoma sebaceum show a distinct pleomorphism. 2. They reveal glial cell-like gene expressions rather than fibroblastic ones in both tissue and cultured condition. 3. In culture their DNA histograms show abnormal patterns, suggesting the presence of nuclei that contain unequal quantities of DNA. 4. Some of these cells show distinct chromosome disarrangement in metaphase followed by abnormal divisions indicating the presence of a functional defect in the "centromere-microtubule-centriole" system. 5. It is characteristic that even in the cloned cells some changes spontaneously occur in the growth and differentiation of cells with an unstable frequency.
Ishibashi
Y
Y
Department of Dermatology, Faculty of Medicine, University of Tokyo, Japan.
Watanabe
R
R
Nogita
T
T
Takahashi
T
T
Onodera
K
K
Kimura
G
G
D000236:Adenoma / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D002455:Cell Division; D002478:Cells, Cultured; D003094:Collagen / Q000648:ultrastructure; D004247:DNA / Q000378:metabolism; D015870:Gene Expression; D005904:Glial Fibrillary Acidic Protein / Q000378:metabolism; D006801:Humans; D066298:In Vitro Techniques; D008854:Microscopy, Electron; D010751:Phosphopyruvate Hydratase / Q000378:metabolism; D012626:Sebaceous Gland Neoplasms / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D014402:Tuberous Sclerosis / Q000235:genetics* / Q000378:metabolism / Q000473:pathology
D005904:Glial Fibrillary Acidic Protein; D003094:Collagen; D004247:DNA; D010751:Phosphopyruvate Hydratase