Summary
Subcutaneous tumors of a patient with v. Recklinghausen's neurofibromatosis contained about 31% collagen calculated on the basis of lipid-free dry weight. Slices of the tumors synthesized collagen at a rate (4.7–8.5% from total protein) which was higher than that of the skin slices (2.8–5.9%). Neurofibromatosis cells were cultured from tumors of two patients. They synthesized relatively much more collagen than cultures of skin fibroblasts of the same patient or of healthy age-matched control persons.
The second patient's cultures were studied in detail. The cell densities of these cultures were higher and expressed more variation than the densities of control skin fibroblasts. Ion exchange cellulose chromatograms, SDS-polyacrylamide gel electrophoresis and 3-hydroxyproline analysis of the radioactive proteins made by the cultures indicate that most of the collagenous proteins resembled type I collagen. High proliferative capacity and high collagen synthesis of selected neurofibromatosis cells explains the growth of solid tumors.
Similar content being viewed by others
References
Abercrombie M (1979) Contact inhibition and malignancy. Nature 281:259–262
Askanas V, Engel WK, Dalakas MC, Lawrence JV, Carter LS (1980). Human Schwann cells in tissue culture. Histochemical and ultrastructural studies. Arch Neurol 37:329–337
Bonner WM, Laskey RA (1974) A film detection method for tritiumlabelled proteins and nucleic acids in polyacrylamide gels. Eur J Biochem 46:83–88
Bornstein P, Sage H (1980) Structurally distinct collagen types. Ann Rev Biochem 49:957–1003
Brockes JP, Raff MC (1979) Studies on cultured rat Schwann cells. II. Comparison with a rat Schwann cell line. In Vitro 15:772–778
Church RL, Tanzer ML, Pfeiffer SE (1973) Collagen and procollagen production by a clonal line of Schwann cells. Proc Natl Acad Sci USA 70:1943–1946
Conley FK, Rubinstein LJ, Spencer AM (1976) Studies on experimental malignant nerve sheath tumors maintained in tissue and organ culture systems. II. Electron microscopy observations. Acta Neuropathol (Berl) 34:293–310
Dehm P, Prockop DJ (1971) Synthesis and extrusion of collagen by freshly isolated cells from chick embryo tendon. Biochim Biophys Acta 240:358–369
Harkin JC, Reed RJ (1969) Tumors of the peripheral nervous system. Atlas of Tumor Pathology, 2nd ser., fasc. 3, Armed Forces Institute of Pathology, Washington, DC
Jalkanen M, Larjava H, Turakainen H, Tammi M (1980) Improved application of the diphenylamine reaction for the determination of DNA. J Biochem Biophys Meth 3:195–199
Juva K, Prockop DJ (1966) Modified procedure for the assay of H3 or C14-labeled hydroxyproline. Anal Biochem 15:77–83
Koeppen AH, Messmore H, Stehbens WE (1971) Interstitial hypertrophic neuropathy. Arch Neurol 24:340–352
Kurkinen M, Alitalo K (1979) Fibronectin and procollagen produced by a clonal line of Schwann cells. FEBS Lett 102:64–68
Lowry OH, Rosebrough NJ, Farr AL, Randall RJ (1951) Protein measurement with the Folin phenol reagent. J Biol Chem 193:265–275
Manner G (1971) Cell division and collagen synthesis in cultured fibroblasts. Exp Cell Res 65:49–60
McFarlane KR, Pollock M, Myers DB (1980) Collagen content in human ulnar nerve. Acta Neuropathol (Berl) 50:217–220
Manthorpe M, Skaper S, Varon S (1980) Purification of mouse Schwann cells using neurite-induced proliferation in serum-free monolayer culture. Brain Res 196:467–482
Penttinen R, Frey H, Aalto M, Vuorio E, Marttala T (1980) Collagen synthesis in cultured cells. In: Viidik A, Vuust J (eds) Biology of collagen. Academic Press, London New York, pp 87–103
Peterkofsky B, Diegelman B (1971) Use of a mixture of proteinase-free collagenases for the specific assay of radioactive collagen in the presence of other proteins. Biochemistry 10:988–994
Pikkarainen J (1968) The molecular structures of vertebrate skin collagens. a comparative study. Acta Physiol Scand (Suppl) 309: 1–72
Pleasure D, Bora FW, Lane J, Prockop D (1974) Regeneration after nerve transection: Effect of inhibition of collagen synthesis. Exp Neurol 45:72–78
Riccardi VM, Maragos VA (1980) The pathophysiology of neurofibromatosis. I. Resistance in vitro to 3-nitrotyrosine as an expression of the mutation. In Vitro 16:706–714
Rubinstein LJ, Conley FK, Hermann MM (1976) Studies on experimental malignant nerve sheath tumors maintained in tissue and organ culture systems. I. Light microscopy observations. Acta Neuropathol (Berl) 34:277–291
Salzer JL, Bunge RP (1980) Studies of Schwann cell proliferation. J Cell Biol 84:739–752
Seyer JM, Kang AH, Whitaker JN (1977) The characterization of type I and type III collagens from human peripheral nerve. Biochim Biophys Acta 492:415–425
Turakainen H, Larjava H, Saarni H, Penttinen R (1980) Synthesis of hyaluronic acid and collagen in skin fibroblasts cultured from patients with osteogenesis imperfecta. Biochim Biophys Acta 628:388–397
Author information
Authors and Affiliations
Additional information
Financially supported by a Research Contract with the Medical Research Council of the Academy of Finland and by a grant from the Finnish Cultural Foundation
Rights and permissions
About this article
Cite this article
Peltonen, J., Marttala, T., Vihersaari, T. et al. Collagen synthesis in cells cultured from v. Recklinghausen's neurofibromatosis. Acta Neuropathol 55, 183–187 (1981). https://doi.org/10.1007/BF00691316
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00691316