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Titolo:
The distribution of PIG-A gene abnormalities in paroxysmal nocturnal hemoglobinuria granulocytes and cultured erythroblasts
Autore:
Noji, H; Shichishima, T; Saitoh, Y; Kai, T; Yamamoto, T; Ogawa, K; Okamoto, M; Ikeda, K; Maruyama, Y;
Indirizzi:
Fukushima Med Univ, Dept Internal Med 1, Fukushima 9601295, Japan Fukushima Med Univ Fukushima Japan 9601295 d 1, Fukushima 9601295, Japan
Titolo Testata:
EXPERIMENTAL HEMATOLOGY
fascicolo: 4, volume: 29, anno: 2001,
pagine: 391 - 400
SICI:
0301-472X(200104)29:4<391:TDOPGA>2.0.ZU;2-B
Fonte:
ISI
Lingua:
ENG
Soggetto:
DECAY-ACCELERATING FACTOR; GLYCOSYLPHOSPHATIDYLINOSITOL-ANCHOR BIOSYNTHESIS; MEMBRANE ATTACK COMPLEX; PERIPHERAL-BLOOD CELLS; GLYCOSYL-PHOSPHATIDYLINOSITOL; APLASTIC-ANEMIA; NORMAL INDIVIDUALS; MOLECULAR-BASIS; FLOW-CYTOMETRY; ERYTHROPOIETIC PRECURSORS;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
71
Recensione:
Indirizzi per estratti:
Indirizzo: Maruyama, Y Fukushima Med Univ, Dept Internal Med 1, 1 Hikariga Oka, Fukushima 9601295, Japan Fukushima Med Univ 1 Hikariga Oka Fukushima Japan 9601295 pan
Citazione:
H. Noji et al., "The distribution of PIG-A gene abnormalities in paroxysmal nocturnal hemoglobinuria granulocytes and cultured erythroblasts", EXP HEMATOL, 29(4), 2001, pp. 391-400

Abstract

Objective. Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hemolytic anemia that is characterized by a deficiency of glycosylphosphatidylinositol anchored membrane proteins due to phosphatidglinositol glycan-class A(PIG-A) gene abnormalities in various lineages of peripheral blood cells and hematopoietic precursors, The purpose of our study was to clarify the distribution of PIG-A gene abnormalities among various cell lineages during differentiation and maturation in PNH patients,Patients and Methods. The expression of CD16b or CD59 in peripheral blood granulocytes or cultured erythroblasts from three Japanese PNH patients wasanalyzed using flow cytometry. PIG-A gene abnormalities in both cell types, including glycophorin A(+) bone marrow erythroblasts, were examined usingnucleotide sequence analysis. The expression study of PIG-A genes from each patient was also performed using JY-5 cells. Results. Flow cytometry revealed that the erythroblasts consisted of negative, intermediate, and positive populations in Cases 1 and 3 and negative and intermediate populations in Case 2. The granulocytes consisted of negative and positive populations in all three cases. DNA sequence analysis indicated that all the PNH cases had two or three types of PIG-A gene abnormalities, and that a predominant clone with an abnormal PIG-A gene was differentin granulocytes and erythroblasts from Cases 2 and 3. Expression studies showed that all the mutations from the patients were responsible for the null phenotype. Conclusion. PIG-A gene abnormalities result in deficiencies of glycosylphosphatidylinositol-anchored proteins in PNH erythroblasts and granulocytes. The distribution of predominant PNH clones with PIG-A gene abnormalities isoften heterogeneous between the cell types, suggesting that a clonal selection of PIG-A gene abnormalities occurs independently among various cell lineages during differentiation and maturation. (C) 2001 International Society for Experimental Hematology. Published by Elsevier Science Inc.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 30/09/20 alle ore 09:43:05