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Biochemical and immunohistochemical studies on overgrown gingival tissues associated with mannosidosis

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Virchows Archiv B

Summary

The gingival tissues of a male patient suffering from mannosidosis and presenting with gingival overgrowth have been studied. Routine histological assessment highlighted the presence of highly enlarged and vacuolated lymphocytes. The morphology of the connective tissues, fibroblasts and epithelium appeared normal. Immunohistochemical staining of the tissues for chondroitin sulfate proteoglycan demonstrated a normal distribution of this component throughout the connective tissues and intense staining associated with the vacuolated lymphocytes. In vitro studies indicated that fibroblasts isolated from the overgrown tissue did not differ from age and sex matched control fibroblasts with respect to proliferation, protein and proteoglycan synthesis. Taken together, these findings imply that the gingvial overgrowth noted in this patient was not due to a defect in the resident fibroblasts but rather reflected a secondary response of the tissues to impaired host defence mechanisms.

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References

  • Autio S, Nordén NE, Ökerman P, Reikinen P, Rapola J, Louhimo T (1973) Mannosidosis: Clinical, fine structural and biochemical findings in three cases. Acta Paediatr Scand 62:555–565

    Article  PubMed  CAS  Google Scholar 

  • Bartold PM (1987) Cyclosporine and gingival overgrowth. J Oral Pathol 16:463–468

    Article  PubMed  CAS  Google Scholar 

  • Bartold PM (1989) Regulation of human gingival fibroblast growth and synthetic activity by cyclosporine-A. J Periodont Res 24:314–321

    Article  PubMed  CAS  Google Scholar 

  • Beaudet AL, Thomas GH (1989) Disorders of glycoprotein degradation. In: Scriver CR, Beaudet AL, Sly WS, Valle D (eds) The metabolic basis if inherited disease. McGraw Hill Information Services Comp, New York, pp 1603–1622

    Google Scholar 

  • Ben Yoseph Y, De Franco CL, Charrow J, Hahn C, Nadler HL (1982) Apparently normal extracellular acidic a-mannosidase in fibroblast cultures from patients with mannosidosis. Am J Hum Genet 34:100–111

    PubMed  CAS  Google Scholar 

  • Carrol D, Dance N, Masson PK, Robinson D, Winchester BG (1972) Human mannosidosis the enzyme defect. Biochim Biophys Acta 49:579–583

    Google Scholar 

  • Cooper A, Sardharwalla IB, Roberts MM (1986) Human-mannosi-dosis deficiency. New Engl J Med 315:1231

    PubMed  CAS  Google Scholar 

  • Couchman JR, Caterson B, Christner JE, Baker JR (1984) Mapping by monoclonal antibody detection of glycosaminoglycans in connective tissues. Nature 307:650–652

    Article  PubMed  CAS  Google Scholar 

  • Daly TD, Wysocki GP, Day CD (1986) Clinical and pharmacologic correlations in cyclosporine-induced gingival hyperplasia. Oral Surg Oral Med Oral Pathol 62:417–421

    Article  Google Scholar 

  • Daniel PF, DeFeudis DF, Lott IT (1981) Mannosidosis: isolation and comparison of mannose-containing oligosaccharides from gingival and urine. Eur J Biochem 114:235–237

    Article  PubMed  CAS  Google Scholar 

  • Desnick RJ, Sharp HL, Gabrowski GA, Brunning RD, Quie PG, Sung JH, Gorlin, RJ, Ikonne JU (1976) Chemical, morphological, immunologic and biochemical studies. Paediatr Res 10:985–996

    Article  CAS  Google Scholar 

  • Emerson TG (1965) Hereditary gingival hyperplasia. A family pedigree of four generations. Oral Surg 19:1–9

    Article  PubMed  CAS  Google Scholar 

  • Fletcher JP (1966) Gingival abnormalities of genetic origin: A preliminary communication with special reference to hereditary generalized gingival fibromatosis. J Dent Res 45:597–612

    Google Scholar 

  • Fratantoni JC, Hall CW, Neufeld EF (1968) The defect in Hurler’s and Hunter’s syndromes: Faulty degradation of mucopolysaccharides. Proc Natl Acad Sci USA 609:649–706

    Google Scholar 

  • Friskopp J, Klintman G (1986) Gingival enlargement. A comparison between cyclosporin and azothioprine treated renal allograft recipients. Swed Dent J 10:85–92

    PubMed  CAS  Google Scholar 

  • Goodman RM and Gorlin RJ (1977) Atlas of the face in genetic disorders. C.V. Mosby Company, St Louis, pp 280–281

    Google Scholar 

  • Gordon BA, Carson R, Haust MD (1980) Unusual clinical and ultrastructural features in a boy with biochemically typical; mannosidosis. Acta Pediatr Scand 69:787–792

    Article  CAS  Google Scholar 

  • Halley DJJ, Winchester BG, Burditt LJ, d’Azzo A, Robinson D, Galjaard H (1980) Comparison of the a-mannosidases in fibroblast cultures from patients with mannosidosis and mucolipidosis II and from controls. Biochem J 187:541–543

    PubMed  CAS  Google Scholar 

  • Hancock LW, Jones MZ, Dawson JG (1986) Glycoprotein metabolism in normal and b-mannosidase-deficient cultured goat and skin fibroblasts. Biochem J 234:175–183

    PubMed  CAS  Google Scholar 

  • Hassell TM, Johnston MC, Dudley KH (1980) Phenytoin-induced teratology and gingival pathology. Raven Press, New York, pp 129–222

    Google Scholar 

  • Hassell TM, Page RC, Narayanan AS, Cooper CG (1976) Diphenyhydantoin (Dilantin) gingival hyperplasia: drug induced abnormality of connective tissue. Proc Natl Acad Sci USA 73:20909–20912

    Article  Google Scholar 

  • Hultberg B, Masson PK (1977) Normal extracellular excretion of acidic mannosidase activity by mannosidosis fibroblast cultures. Biochim Biophys Acta 481:537–577

    Google Scholar 

  • Johnson BD, El-Guindy M, Ammons WF, Naryanan AS, Page RC (1986) A defect in fibroblasts from an unidentified syndrome with gingival hyperplasia as the predominant feature. J Periodont Res 21:403–413

    Article  PubMed  CAS  Google Scholar 

  • Jones MZ, Dawson G (1981) Caprine-mannosidosis. Inherited deficiency of b-D-mannosidase. J Biol Chem 256:5185–5188

    PubMed  CAS  Google Scholar 

  • Kistler JP, Lott IT, Kolodny EH, Friedman RB, Nersasian R, Schnur J, Mihm MC, Dvorak AM, Dickersiin R (1977) Mannosidosis. New clinical presentation, enzyme studies and carbohydrate analysis. Arch Neurol 34:45–51

    PubMed  CAS  Google Scholar 

  • Lederman D, Lumerman H, Reuben J, Freedman P (1984) Gingival hyperplasia associated with Nifedipine therapy. Oral Surg Oral Med Oral Pathol 57:620–622

    Article  PubMed  CAS  Google Scholar 

  • Little TM, Girgis SS, Masotti RE (1975) Diphenyhydantoin-induced gingival hyperplasia — its response to changes in drug dosage. Dev Med Child Neurol 17:421–424

    PubMed  CAS  Google Scholar 

  • Loeb H, Tondeur M, Toppet M, Cremer N (1969) Clinical, biochemical and ultrastructural studies of an atypical form of mucopolysaccharidosis. Acta Paediat Scand 58:220–228

    Article  PubMed  CAS  Google Scholar 

  • Marsh CA, Goulay GC (1971) Evidence for a non-lysosomal amannosidase in rat liver homogenates. Biochim Biophys Acta 235:142–148

    PubMed  CAS  Google Scholar 

  • Matthews N, Neale ML (1987) Cytotoxicity assays for tumor necrosis factor and lymphotoxin. In: Clemens MJ, Morris AG, Gearing AJH (eds) Lymphokines and interferons. A Practical Approach. IRL Press, Oxford, pp 221–225

    Google Scholar 

  • Mitchell ML, Erickson RP, Schmid D, Hieber V, Poznanski AK, Hicks SP (1981) Mannosidosis: two brothers with different degrees of disease severity. Clin Genet 20:191–202

    PubMed  CAS  Google Scholar 

  • Mónus Z, Konyár E, Szabó L (1977) Histomorphologic and histochemical investigations in mannosidosis. A light and electron microscopic study. Virchows Archiv [B] 26:159–73

    Google Scholar 

  • Narayanan AS, Page RC (1976) Biochemical characterization of collagens synthesized by fibroblasts derived from normal and diseased human gingival. J Biol Chem 251:5464–5471

    PubMed  CAS  Google Scholar 

  • Nolan CM, Sly WS (1989) I-cell disease and pseudo-Hurler polysystrophy. Disorders of lysosomal enzyme phosphorylation and localization. In: Scriver CR, Beaudet AL, Sly WS, Valle D. The metabolic basis of inherited disease. McGraw Hill Information Serv, New York, pp 1589–1681

    Google Scholar 

  • Pökerman PA (1967) A generalized storage disorder resembling Hurler’s syndrome. Lancet 2:239–241

    Google Scholar 

  • Oohira A, Matsui F, Oki T, Nogami H (1987) Deficiency of density dependent regulation of cell growth in the culture of skin fibroblasts from patients with mucolipidosis III. J Cell Sci 87:249–257

    PubMed  Google Scholar 

  • Patton MA, Barnes IC, Young ID, Harper PS, Pennock CA (1982) Mannosidosis in two brothers: prolonged survival in the severe phenotype. Clin Genet 22:284–289

    Article  PubMed  CAS  Google Scholar 

  • Quie PG, Cates KL (1977) Clinical conditions associated with defective polymorphonuclear leukocyte chemotaxis. Am J Pathol 88:711–726

    PubMed  CAS  Google Scholar 

  • Rapraeger A, Yeaman C (1989) A quantitative solid phase assay for identifying radiolabelled glycosaminoglycans in crude cell extracts. Anal Biochem 179:361–365

    Article  PubMed  CAS  Google Scholar 

  • Spranger J, Gehler J, Cantz M (1976) The radiographie features of mannosidosis. Radiology 119:410–417

    Google Scholar 

  • Tabas I, Kornfeld S (1979) Purification and characterization of a-mannosidase capable of processing asparagine-linked oligosaccharides. J Biol Chem 254:11655–11663

    PubMed  CAS  Google Scholar 

  • Taylor HA, Thomas GH, Aylsworth A, Stevenson RE, Reynolds LW (1975) Mannosidosis: deficiency of a specific a-mannosidase component in cultured fibroblasts. Clin Chim Acta 59:93–99

    Article  PubMed  CAS  Google Scholar 

  • Tsvetkova IV, Rosenfeld EL, Prigizina IG (1980) An unusual case of mannosidosis with severe deficiency of acid mannosidase in leukocytes and high residual enzymatic activity in skin fibroblasts. Clin Chim Acta 107:37–42

    Article  PubMed  CAS  Google Scholar 

  • van Pelt J, Hokke CH, Dorland L, Duran M, Kamerlin JP, Vliegenthart FG (1990) Accumulation of mannosyl-b-(l-4)-N-acetylglucosamine in fibroblasts and leukocytes of patients with a deficiency of b-mannosidase. Clin Chim Acta 187:55–60

    Article  PubMed  Google Scholar 

  • Wahl SM, Wahl LM, McCarthy JB (1979) Lymphocyte-mediated activation of fibroblast proliferation and collagen production. J Immunol 121:942–946

    Google Scholar 

  • Wenger DA, Sujansky E, Fennessey PV, Thompson TN (1986) Human b-mannosidosis deficiency. New Engl J Med 315:1201–1231

    PubMed  CAS  Google Scholar 

  • Yunis JJ, Lewandowski RC, Sanfilippo SJ, Tsai MY, Foni I, Bruhl HH (1976) Clinical manifestations of mannosidosis. A longitudinal study. Am J Med 61:841–848

    Article  PubMed  CAS  Google Scholar 

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Bartold, P.M. Biochemical and immunohistochemical studies on overgrown gingival tissues associated with mannosidosis. Virchows Archiv B Cell Pathol 62, 391–399 (1992). https://doi.org/10.1007/BF02899709

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