Important Genes in the Pathogenesis of 5q- Syndrome and Their Connection with Ribosomal Stress and the Innate Immune System PathwayReport as inadecuate




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Leukemia Research and TreatmentVolume 2012 2012, Article ID 179402, 14 pages

Review Article

Institute of Hematology and Blood Transfusion, U Nemocnice 1, 128 20 Prague 2, Czech Republic

Center of Experimental Hematology, First Medical Faculty, Charles University, Institute of Pathological Physiology, 128 53 Prague 2, Czech Republic

Received 25 September 2011; Revised 6 November 2011; Accepted 14 November 2011

Academic Editor: Daniela Cilloni

Copyright © 2012 Ota Fuchs. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Myelodysplastic syndrome MDS with interstitial deletion of a segment of the longarm of chromosome 5q del5q is characterized by bone marrow erythroid hyperplasia,atypical megakaryocytes, thrombocythemia, refractory anemia, and low risk of progression toacute myeloid leukemia AML compared with other types of MDS. The long arm ofchromosome 5 contains two distinct commonly deleted regions CDRs. The more distal CDRlies in 5q33.1 and contains 40 protein-coding genes and genes coding microRNAs miR-143,miR-145. In 5q-syndrome one allele is deleted that accounts for haploinsufficiency of thesegenes. The mechanism of erythroid failure appears to involve the decreased expression of theribosomal protein S14 RPS14 gene and the upregulation of the p53 pathway by ribosomalstress. Friend leukemia virus integration 1 Fli1 is one of the target genes of miR145. Increased Fli1 expression enables effective megakaryopoiesis in 5q-syndrome.





Author: Ota Fuchs

Source: https://www.hindawi.com/



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