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Titolo:
RECENT ADVANCES IN PAROXYSMAL-NOCTURNAL HEMOGLOBINURIA - FROM THE BIOLOGY TO THE CLINIC
Autore:
SOCIE G;
Indirizzi:
HOP ST LOUIS,GREFFE MOELLE & UNITE RECH BIOL CELLULES SOUCHES,SERV HEMATOL F-75475 PARIS 10 FRANCE
Titolo Testata:
HEMATOLOGY AND CELL THERAPY
fascicolo: 4, volume: 39, anno: 1997,
pagine: 175 - 187
SICI:
1269-3286(1997)39:4<175:RAIPH->2.0.ZU;2-1
Fonte:
ISI
Lingua:
ENG
Soggetto:
DECAY-ACCELERATING FACTOR; PIG-A GENE; BONE-MARROW TRANSPLANTATION; GLYCOSYLPHOSPHATIDYLINOSITOL-ANCHOR BIOSYNTHESIS; PROTEIN TYROSINE KINASES; PERIPHERAL-BLOOD CELLS; SEVERE APLASTIC-ANEMIA; BUDD-CHIARI SYNDROME; PHOSPHATIDYLINOSITOL-LINKED PROTEINS; COMPLEMENT REGULATORY PROTEIN;
Keywords:
PAROXYSMAL NOCTURNAL HEMOGLOBINURIA; PIG-A GENE; GPI ANCHOR; APLASTIC ANEMIA;
Tipo documento:
Review
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
181
Recensione:
Indirizzi per estratti:
Citazione:
G. Socie, "RECENT ADVANCES IN PAROXYSMAL-NOCTURNAL HEMOGLOBINURIA - FROM THE BIOLOGY TO THE CLINIC", HEM CELL TH, 39(4), 1997, pp. 175-187

Abstract

PNH is now known as an acquired, clonal disorder of the hematopietic stem cells caused by somatic mutation in the X-linked PIG-A gene encoding a protein involved in the synthesis of the glycosylphosphatidylinositol (GPI) anchor by which many proteins are attached to the membrane. Since the past few years, significant advances in the knowledge of the biology of this rare disease have been done. Similarily on the clinical ground, large series of patients with PNH have been published recently, providing estimates of factors affecting survival and of long term follow-up of significant numbers of patients. In this overview we focus on recent advances in the biology and the clinical aspects of this disease, and more importantly try to underline the numerous aspectsof yet un-answered questions.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 01/04/20 alle ore 11:26:25