A new genetic isolate of gray platelet syndrome (GPS): Clinical, cellular, and hematologic characteristics

Molecular Genetics and Metabolism

Volumn 74, Issue 3, 2001, Pages 303-313

  Falik Zaccai, Tzipora C ^a   Anikster, Yair ^b   Rivera, Candido E ^c   Horne III, McDonald K ^c   Schliamser, Liliana ^d   Phornphutkul, Chanika ^b   Attias, Dina ^d   Hyman, Tehila ^e   White, James G ^f   Gahl, William A ^b  

^a TECHNION ISRAEL INSTITUTE OF TECHNOLOGY   (Israel)

^b EUNICE KENNEDY SHRIVER NATIONAL INSTITUTE OF CHILD HEALTH AND HUMAN DEVELOPMENT   (United States)

^c NATIONAL INSTITUTES OF HEALTH   (United States)

^d BNAI ZION MEDICAL CENTER   (Israel)

^e NATIONAL CANCER INSTITUTE   (United States)

^f UNIVERSITY OF MINNESOTA MEDICAL SCHOOL   (United States)

Author keywords

Fibrinogen receptor; Genetic isolate; Gray platelet syndrome; P selectin

Indexed keywords

ADENOSINE DIPHOSPHATE; ANTIGEN; FIBROBLAST GROWTH FACTOR; PADGEM PROTEIN; THROMBIN RECEPTOR ACTIVATING PEPTIDE; VON WILLEBRAND FACTOR;

ARTICLE; BONE MARROW EXAMINATION; CASE REPORT; CHILD; CLINICAL FEATURE; ELECTRON MICROSCOPY; EMPERIPOLESIS; FEMALE; GENETIC ANALYSIS; GRAY THROMBOCYTE SYNDROME; HEMATOPOIESIS; HEMATOPOIETIC CELL; HISTOPATHOLOGY; HUMAN; MALE; MEGAKARYOCYTE; MYELOFIBROSIS; PATHOGENESIS; PRIORITY JOURNAL; SYNDROME; THROMBOCYTE ACTIVATION; THROMBOCYTE AGGREGATION; THROMBOCYTE ANOMALY;

EID: 0035196989     PISSN: 10967192     EISSN: None     Source Type: Journal    
DOI: 10.1006/mgme.2001.3247     Document Type: Article

References (34)

1. Gray platelet syndrome: A variety of qualitative platelet disorder
2. Megakaryocytes and platelets in alpha-granule disorders
3. Gray platelet syndrome with splenomegaly and signs of extramedullary hematopoiesis: A case report with review of the literature
4. Inherited disorders of platelet α-granules
5. Platelet α-granules
6. Pathologic interaction between megakaryocytes and polymorphonuclear leukocytes in myelofibrosis
7. Gray platelet syndrome in the elderly
8. Alpha-granule pool of glycoprotein IIb-IIIa in normal and pathologic platelets and megakaryocytes
9. Platelet alpha granule deficiency associated with decreased P. selectin and selective impairment of thrombin-induced activation in a new patient with gray platelet syndrome (alpha-storage pool deficiency)
10. Deficiency of P-selectin in a patient with grey platelet syndrome
11. Gray platelet syndrome as a rare cause of myelofibrosis
12. Platelet von Willebrand factor: Comparison with plasma von Willebrand factor
13. Platelet immunoglobuin G: Its significance for the evaluation of thrombocytopenia and for understanding the origin of alpha-granule proteins
14. Glycocalicin levels in the plasma of HIV+ patients: An indicator of platelet turnover
15. Interaction of membrane systems in blood platelets
16. Biochemical studies of two patients with the gray platelet syndrome: Selective deficiency of platelet alpha granules
17. Gray platelet syndrome (GPS) in a native American female
18. The gray platelet syndrome in four members of a Palestinian Arab family
19. Anesthetic complications of the gray platelet syndrome
20. Gray platelet syndrome
21. Electron-microscopic and functional studies on platelets in gray platelet syndrome
22. Simultaneous occurrence of gray platelet syndrome and idiopathic pulmonary fibrosis: A role for abnormal megakaryocytes in the pathogenesis of pulmonary fibrosis?
23. Defective α-granule production in megakaryocytes from gray platelet syndrome
24. Megakaryocytes and myelofibrosis in gray platelet syndrome
25. Emperipolesis of granular leukocytes within megakaryocytes in human hemopoietic bone marrow
26. On the alleged phagocytosis by megakaryocytes
27. Synthesis and secretion of von Willebrand factor and fibronectin in megakaryocytes at different phases of maturation
28. 3 (glycoprotein IIb-IIIa)
29. Gray platelet syndrome: Demonstration of alpha granule membranes that can fuse with the cell surface
30. Biochemical studies of two patients with the gray platelet syndrome
31. A platelet alpha-granule membrane protein (GMP-140) is expressed on the plasma membrane after activation
32. Resting platelets contain a substantial centrally located pool of glycoprotein IIb-IIIa complex which may be accessible to some but not other extracellular proteins
33. Distribution of ligand-occupied alpha IIb beta 3 in resting and activated human platelets determined by expression of a novel class of ligand-induced binding site recognized by monoclonal antibody AP6
34. Gray platelet syndrome: Selective alpha-granule deficiency and thrombocytopenia due to increased platelet turnover