Murine models of paroxysmal nocturnal hemoglobinuria

Annals of the New York Academy of Sciences

Volumn 963, Issue , 2002, Pages 290-296

  Rosti, Vittorio ^a  

^a FONDAZIONE IRCCS POLICLINICO SAN MATTEO   (Italy)

Author keywords

Cre loxP; Knockout mice; Paroxysmal nocturnal hemoglobinuria; Phosphatidyl inositol glycan complementation group A; PIG A

Indexed keywords

GLYCAN; GLYCOSYLPHOSPHATIDYLINOSITOL; MEMBRANE PROTEIN; PHOSPHATIDYLINOSITOL;

ANIMAL EXPERIMENT; ANIMAL MODEL; BIOSYNTHESIS; BONE MARROW DEPRESSION; CELL MUTANT; CELL PROLIFERATION; CLINICAL EXAMINATION; CLINICAL FEATURE; CONFERENCE PAPER; CONTROLLED STUDY; CYTOPENIA; DISEASE COURSE; GENE; GENE DISRUPTION; GENE TARGETING; GENETIC COMPLEMENTATION; INTRAVASCULAR HEMOLYSIS; KNOCKOUT MOUSE; MOUSE; NONHUMAN; PAROXYSMAL NOCTURNAL HEMOGLOBINURIA; PIG A GENE; SOMATIC MUTATION; STEM CELL; SYMPTOM; THROMBOSIS;

ANIMALIA; MURINAE; SUS SCROFA;

EID: 0036291105     PISSN: 00778923     EISSN: None     Source Type: Book Series    
DOI: 10.1111/j.1749-6632.2002.tb04120.x     Document Type: Conference Paper

References (40)

1. Paroxysmal nocturnal haemoglobinuria: Clinical manifestations, haematology and nature of the disease
2. Natural history of paroxysmal nocturnal haemoglobinuria
3. Paroxysmal nocturnal haemoglobinuria: Long term follow up and prognostic factors
4. Allogeneic bone marrow transplantation for paroxysmal nocturnal hemoglobinuria
5. The cloning of PIG-A, a component in the early step of GPI-anchor biosynthesis
6. Paroxysmal nocturnal haemoglobinuria (PNH) is caused by somatic mutations in the PIG-A gene
7. Specific defect in N-acetylglucosammine incorporation in the biosynthesis of the glyosylphosphatidylinositol anchor in cloned cell lines in patients with paroxysmal nocturnal haemoglobinuria
8. Genetics of PNH
9. Somatic mutations and cellular selection in paroxysmal nocturnal haemoglobinuria
10. A patient with paroxysmal nocturnal hemoglobinuria bearing four independent PIG-A mutant clones
11. Hemolysis in PNH
12. Pathophysiology of paroxysmal nocturnal haemoglobinuria
13. Resistance to apoptosis caused by PIG-A gene mutations in paroxysmal nocturnal hemoglobinuria
14. Decreased number of circulating BFU-E in paroxysmal nocturnal haemoglobinuria
15. Impaired hematopoiesis in parosxysmal nocturnal haemoglobinuria/aplastic anemia is not associated with a selective proliferative defect in the glycosylphosphatidylinositol-anchored protein-deficient clone
16. Apoptosis resistance of blood cells from patients with paroxysmal nocturnal hemoglobinuria, aplastic anemia and myelodisplastic syndrome
17. The PIG-A mutation and absence of glycosylphosphatidylinositol-linked proteins do not confer resistance to apoptosis in paroxysmal nocturnal hemoglobinuria
18. Paroxysmal nocturnal hemoglobinuria
19. The dual pathogenesis of paroxysmal nocturnal hemoglobinuria
20. Late haematological complications in severe aplastic anaemia
21. A pathogenetic link between aplastic and paroxysmal nocturnal hemoglobinuria is suggested by a high frequency of plastic anemia patients with a deficiency of phosphatidylinositol glycan anchored proteins
22. Somatic mutations and clonal selection in the pathogenesis and in the control of paroxysmal nocturnal hemoglobinuria
23. The pathophysiology of acquired aplastic anemia
24. Emergence of CD52-, phosphatidylinositol-glycan-anchor-deficient T lymphocytes after in vivo application of Campath-1H for refeactory B-cell non-Hodgkin's lymphoma
25. Antibody selection against CD52 produces a paroxysmal nocturnal haemoglobinuria phenotype in human lymphocytes by a novel mechanism
26. The PNH phenotype cells that emerge in most patients after Campath-1H therapy are present prior to treatment
27. Clonal population of hemopoietic cells with paroxysmal nocturnal hemoglobinuria genotype and phenotype are present in normal individuals
28. Impaired growth and elevated Fas receptor expression in PIG-A+ stem cells in primary paroxysmal nocturnal hemoglobinuria
29. The new mouse genetics: Altering the genome by gene targeting
30. Geno ko technology: A methodological overview for the interested novice
31. A knock-out model of paroxysmal nocturnal hemoglobinuria: Pig-a- hematopoiesis is reconstituted following intercellular transfer of GPI-anchored proteins
32. Glycosylphosphatidyl inositol-anchor-deficient mice: Implications for clonal dominance of mutant cells in paroxysmal nocturnal hemoglobinuria
33. Murine embryonic stern cells without pig-a gene activity are competent for hematopoiesis with the PNH phenotype but not for clonal expansion
34. Different roles of glycosylphosphatidyl inositol in various hematopoietic cells as revealed by a mouse model of paroxysmal nocturnal hemoglobinuria
35. Increased sensitivity to complement and a decrease red blood cells life span in mice mosaic fora non functional Pig-a gene
36. Functional competence of T cells in the absence of glycosylphosphatidylinositol-anchored proteins caused by T cell-specific disruption of the Pig-a gene
37. Phenotypic and functional analysis of lymphocytes in paroxysmal nocturnal hemoglobinuria
38. FES-Cre targets phosphatidylinositol Glycan class A (PIGA) inactivation to hematopoitic stem cell in the bone marrow
39. GATA1-Cre mediates Piga gene inactivation in the erythroid/megakaryocitic lineage and leads to circulating red cells with a partial deficiency in glycosylphosphatidylinositol-linked proteins (paroxysmal nocturnal hemoglobinuria type II cells)