Summary
The distribution of glial fibrillary acidic protein (GFAP) in the central nervous system (CNS) lesions of tuberous sclerosis (TS) was examined using antiserum against GFAP and the peroxidase antiperoxidase method of Sternberger. In cortical tubers there were islands of gemistocytic astrocytes staining intensely for GFAP and occasional giant cells having some cytoplasmic staining. The majority of the cortical giant cells had no GFAP. The islands were separated by areas devoid of astrocytes with perikaryal staining. A faintly staining fibrous network was found between these islands. The majority of cells in the subependymal nodules stained. The retinal phakoma stained but not as intensely as the subependymal nodules. There was no staining whatsoever in the giant cell subependymal tumors. Absence of GFAP staining in the subependymal giant cell tumors makes their classification as astrocytomas less certain.
Similar content being viewed by others
References
Arseni C, Alexianu M, Horvat L, Alexianu D, Petrovici AL (1972) Fine structure of atypical cells in tuberous sclerosis. Acta Neuropathol (Berl) 21:185–193
Choi BH, Lapham LW (1978) Radial glia in human fetal cerebrum. A combined Golgi, immunofluorescent and electron microscopic study. Brain Res 148:295–311
Critchley M, Earl CJC (1932) Tuberous sclerosis and allied conditions. Brain 55:311–346
De Chadarévian JP, Hollenberg RD (1979) Subependymal giant cell tumor of tuberous sclerosis: A light and ultrastructural study. J Neuropathol Exp Neurol 38:419–433
De Vries GH, Eng LF, Lewis DH, Hadfield MG (1976) The protein composition of bovine myelin-free axons. Biochim Biophys Acta 439:133–145
Deck JHN, Eng LF, Bigbee J, Woodcock SM (1978) The role of glial fibrillary acid protein in the diagnosis of central nervous system tumors. Acta Neuropathol (Berl) 42:183–190
Eng LF, De Vries GH, Lewis DH, Bigbee JW (1976) Specific antibody to major 47,000 M.W. protein fraction of bovine myclin-free axons. Fed Proc 35:1766
Eng LF, Rubinstein LJ (1978) Contribution of immunohistochemistry to diagnostic problems of human cerebral tumors. J Histochem Cytochem 26:513–522
Globus JH, Strauss I, Selinsky H (1932) Das Neurospongioblastom, eine primäre Gehirngeschwulst bei disseminierter Neurospongioblastose (Tuberöse Sklerose). Z Ges Neurol Psychiat 140:1–29
Hauser OW, McLeod RA (1979) Roentgenographic experience at the Mayo Clinic. In: Gomez M (ed) Tuberous sclerosis. Raven Press, New York, pp 27–53
Kepes JJ, Rubinstein LJ, Eng LF (1979) Pleomorphic xanthoastrocytoma: A distinctive meningocerebral glioma of young subjects with relatively favorable prognosis. Cancer 44:1839–1852
Kuntz NL, Gomez MR (1979) Genetics, population studies and pathogenesis. In: Gomez M (ed) Tuberous sclerosis. Raven Press, New York, pp 207–220
Ludwin SK, Kosek JC, Eng LF (1976) The topographical distribution of S-100 and GFA proteins in the adult rat brain: An immunohistochemical study using horseradish peroxidaselabeled antibodies. J Comp Neurol 165:197–208
Regan TJ (1979) Neuropathology. In: Gomez M (ed) Tuberous sclerosis. Raven Press, New York, pp 6–83
Ribadeau Dumas JL, Poirier J, Escourolle R (1973) Etude ultrastructurales des lesions cerebrales de la sclerose tubereuse de Bourneville. Acta Neuropathol (Berl) 25:259–270
Russell DS, Rubinstein LJ (1977) Pathology of tumors of the nervous system, 4th edn. Williams and Wilkins, Baltimore, pp 165
Schachner M, Hedley-Whyte ET, hsu DW, Schoomaker G, Bignami A (1977) Ultrastructural localization of glial fibrillary acidic protein in mouse cerebellum by immunoperoxidase labeling. J Cell Biol 75:67–73
Sternberger LA (1979) Immunocytochemistry. 2nd edn. Wiley, New York
Vaughn JE, Peters A (1976) The morphology and development of neurologial cells. In: Cellular aspects of neural growth and differentiation. University of California Press, Berkley, pp 103–140
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Stefansson, K., Wollmann, R. Distribution of glial fibrillary acidic protein in central nervous system lesions of tuberous sclerosis. Acta Neuropathol 52, 135–140 (1980). https://doi.org/10.1007/BF00688011
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00688011