Thromb Haemost 1985; 54(04): 862-865
DOI: 10.1055/s-0038-1660149
Original Article
Schattauer GmbH Stuttgart

Evidence for Glycoprotein Abnormality in Platelets from Patients with May-Hegglin Anomaly

Giorgio Ricci
1   The Istituto di Clinica Medica, Italy
,
Roberto Manservigi
2   The Istituto di Microbiologia dell Universita di Ferrara, Ferrara, Italy
,
Loredana Albonici
2   The Istituto di Microbiologia dell Universita di Ferrara, Ferrara, Italy
,
Giorgio Zavagli
3   The Istituto di Patologia Medica, Italy
,
Enzo Cassai
2   The Istituto di Microbiologia dell Universita di Ferrara, Ferrara, Italy
› Author Affiliations
Further Information

Publication History

Received 01 April 1985

Accepted 27 September 1985

Publication Date:
19 July 2018 (online)

Summary

In the present research protein analysis on SDS-polyacry-lamide gel electrophoresis (PAGE) has been used to study the glycoprotein pattern of the blood platelets of four members from a family affected by May-Hegglin anomaly. In order to characterize the glycoprotein fractions, lactoperoxidase-iodination and immunoprecipitation procedures were used.

N,N’diallyltartardiamide (DATD) cross-linked gel electrophoresis was shown to improve the glycoprotein pattern resolution, although with the lactoperoxidase-iodination the glycoprotein characterization of May-Hegglin platelets overlapped the normal. On the other hand with the immunoprecipitation the specific antiserum precipitated all the five major fractions of normal membrane glycoproteins, but it was shown to react quite poorly with the component V of the May-Hegglin glycoprotein pattern.

 
  • References

  • 1 Hegglin R. Uber eine besondere Form gleichzeitiger konstitutioneller Veränderungen der Neutrophylen und Thrombozyten (kon-stitutionelle polyphyle Reifungstorung). Arch Klaus-Stift Vererb-Forsch 1945; 20: 1-13
  • 2 Oski FA, Naiman JL, Allen DM, Diamond LK. Leucocyte inclusions - Döhle bodies - associated with platelet abnormality (the May-Hegglin anomaly). Report of a family and review of the literature. Blood 1962; 20: 657-667
  • 3 Buchanan JC, Pearce L, Wetherley-Mein G. The May-Hegglin anomaly. A family report and chromosome study. Br J Haematol 1964; 10: 508-512
  • 4 Volpe E, Cuccurullo L, Valente A, Jori GP, Buonanno G. The May-Hegglin anomaly. Further study on leucocyte inclusions and platelet ultrastructure. Acta Haematol 1974; 52: 238-247
  • 5 Mayer M, Sperling H, Schaefer J, Qeisser W. Megacariocyte poly-ploidization in May-Hegglin anomaly. Acta Haematol 1978; 60: 42-52
  • 6 Djaldetti M, Creter D, Bujanover Y, Elian E. Ultrastructural and functional studies of the platelets in patients with May-Hegglin anomaly. Haematologica 1982; 67: 530-538
  • 7 Okumura T, Jamieson GA. Platelet glycocalycin. Orientation of glycoproteins of the human platelet surface. J Biol Chem 1976; 251: 5944-5949
  • 8 Phillips DR, Poh Agin P. Platelet plasma membrane glycoproteins. Evidence for the presence of non equivalent disulfide bonds using non reduced-reduced two dimensional gel electrophoresis. J Biol Chem 1977; 252: 2121-2126
  • 9 Phillips DR. Surface labelling as a tool to determine structure-function relationships of platelet plasma membrane glycoproteins. Thromb Haemostas 1979; 42: 1638-1651
  • 10 Phillips DR. Effect of trypsin on the exposed polypeptides and glycoproteins in the human platelet membrane. Biochemistry 1972; 11: 4582-4587
  • 11 Jenkins CS P, Clemetson KJ. Membrane glycoproteins in normal and pathological platelets. In: Recent Progress in Cell Biology: Leukocytes and Platelets Ulutin ON. ed Bibliotheca Haematologica; 14-19 1978
  • 12 Jenkins CS P, Ali-Briggs EF, Zonneveld GTE, Sturk A, Clemetson KJ. Human blood platelet membrane glycoproteins - Resolution in different polyacrylamide gel electrophoretic systems. Thromb Haemostas 1979; 42: 1490-1502
  • 13 Phillips DR, Poh Agin P. Platelet membrane defects in Glanzmann’s thrombasthenia. Evidence for decreased amounts of two major glycoproteins. J Clin Invest 1977; 60: 535-545
  • 14 Hagen I, Nurden A, Bjerrum OI, Solum NO, Caen J. Immunochemical evidence for protein abnormalities in platelets from patients with Glanzmann’s thrombasthenia and Bemard-Soulier syndrome. J Clin Invest 1980; 65: 722-731
  • 15 McEver RP, Baenziger NL, Majerus PW. Isolation and quantitation of the platelet membrane glycoprotein deficient in thrombasthenia using a monoclonal hybridoma antibody. J Clin Invest 1980; 65: 1311-1318
  • 16 Caen JP, Nurden AP, Jeanneau C, Michel H, Toblem G, Levy-Toledano S, Sultan JN, Valensi F, Bernard J. Bernard-Soulier syndrome: A new platelet glycoprotein abnormality. Its relationship with platelet adhesion to subendothelium and with factor VIII von Willebrand protein. J Lab Clin Med 1976; 87: 586-596
  • 17 Gerrard JM, Schollmeyer JV, Phillips DR, White JG. Platelet glycoprotein III and alpha-actin: possible identity and deficiency in thrombasthenia. Fed Proc 1978; 37: 407
  • 18 Heine JW, Honess RW, Cassai E, Roizman B. Proteins specified by Herpes simplex virus XII. The virion polypeptides of type 1 strains. J Virol 1974; 14: 640-651
  • 19 Para MF, Baucke RB, Spear PG. Glycoprotein gE of Herpes simplex virus type 1: Effects of anti-gE on virion infectivity and on virus-induced Fc-binding receptors. J Virol 1982; 41: 129-136
  • 20 Dacie JV, Lewis SM. Staining methods. In: Practical Haematology Churchill Livingstone; 70-72 Edinburgh: 1975
  • 21 Massini P, Lüscher EF. Some effects of ionophores for divalent cations on blood platelets. Comparison with the effects of thrombin. Biochim Biophys Acta 1974; 109-121
  • 22 Born GV R, Cross MJ. The aggregation of blood platelets. J Physiol 1963; 168: 178-195
  • 23 Proctor RR, Rapaport SI. The partial thromboplastin time with kaolin. A simple screening test for first stage plasma clotting factor deficiencies. Am J Clin Pathol 1961; 36: 212-219
  • 24 Clauss A. Gerinnungsphysiologische Schnellmethode zur Bestimmung des Fibrinogens. Acta Haematol 1957; 17: 237-245
  • 25 Allington MJ. Detection of fibrinogen degradation products by a latex clumping method. Scand J Haematol 1971; 8: 115-119
  • 26 Phillips DR, Poh Agin P. Platelet plasma membrane glycoproteins. Identification of a proteolytic substrate for thrombin. Biochem Biophys Res Commun 1977; 75: 940-947