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Titolo:
Proliferative capacity of single isolated CD34(+) hematopoietic stem/progenitor cells in paroxysmal nocturnal hemoglobinuria
Autore:
Han, B; Wu, YJ; Lu, ZJ; Zhang, ZN;
Indirizzi:
Peking Union Med Coll Hosp, Div Hematol, Dept Med, Chinese Acad Med Sci, Beijing 100730, Peoples R China Peking Union Med Coll Hosp Beijing PeoplesR China 100730 eoples R China
Titolo Testata:
INTERNATIONAL JOURNAL OF HEMATOLOGY
fascicolo: 1, volume: 74, anno: 2001,
pagine: 42 - 52
SICI:
0925-5710(200107)74:1<42:PCOSIC>2.0.ZU;2-2
Fonte:
ISI
Lingua:
ENG
Soggetto:
HUMAN-BONE-MARROW; PIG-A GENE; SEVERE APLASTIC-ANEMIA; STEM-CELLS; PROGENITOR CELLS; ADHESION MOLECULES; PERIPHERAL-BLOOD; PATHOGENETIC LINK; SURFACE-PROTEINS; FLOW-CYTOMETRY;
Keywords:
paroxysmal nocturnal hemoglobinuria; hematopoietic stem cell; single-cell culture;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Citazioni:
49
Recensione:
Indirizzi per estratti:
Indirizzo: Han, B Peking Union Med Coll Hosp, Div Hematol, Dept Med, Chinese Acad MedSci, Beijing 100730, Peoples R China Peking Union Med Coll Hosp Beijing Peoples R China 100730 R China
Citazione:
B. Han et al., "Proliferative capacity of single isolated CD34(+) hematopoietic stem/progenitor cells in paroxysmal nocturnal hemoglobinuria", INT J HEMAT, 74(1), 2001, pp. 42-52

Abstract

Paroxysmal nocturnal hemoglobinuria (PNH) results from somatic mutations of the X-linked PIG-A (phosphatidylinositol glycan-class A) gene, which occurs on a hematopoietic stem cell level, leading to a proportion of blood cells being deficient in all glycosylphosphatidylinositol (GPI)-anchored surface proteins. Although these GPI-deficient cells can explain many of the clinical symptoms of PNH, the pathogenesis of PNH is still somewhat obscure and many questions remain. To assess the hematopoietic defect involved in PNH, CD34(+)CD59(+) (normal phenotype hematopoietic stem/progenitor) and CD34()CD59(+) (PNH phenotype) cells from PNH patients (n = 16) and CD34(+)CD59() cells from healthy volunteers (n = 10) were sorted as single cells into 96-well flat-bottom culture plates containing culture medium supplemented with stem cell factor, interleukin (IL)-3, erythropoietin, granulocyte-macrophage-colony-stimulating factor (GM-CSF), G-CSF, IL-6, thrombopoietin, and Flt-3 ligand. We found that the single PNH CD34(+)CD59(+) cells had a growth advantage over the single CD34(+)CD59(+) cells to some extent, but they both had impaired growth abilities compared with CD34(+) cells from healthy volunteers. (C) 2001 The Japanese Society of Hematology.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 06/04/20 alle ore 00:59:10