Errant innate immune signaling in del(5q) MDS.

نویسنده

  • Daniel T Starczynowski
چکیده

In this issue of Blood, Keerthivasan et al have identified that the deletion of mDia1, a chromosome 5q gene, contributes to myelodysplastic syndromes (MDSs) by increasing innate immune signaling in granulocytes.

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Loss of Tifab, a del(5q) MDS gene, alters hematopoiesis through derepression of Toll-like receptor–TRAF6 signaling

TRAF-interacting protein with forkhead-associated domain B (TIFAB) is a haploinsufficient gene in del(5q) myelodysplastic syndrome (MDS). Deletion of Tifab results in progressive bone marrow (BM) and blood defects, including skewed hematopoietic stem/progenitor cell (HSPC) proportions and altered myeloid differentiation. A subset of mice transplanted with Tifab knockout (KO) HSPCs develop a BM ...

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Partial or complete deletion of the long arm of chromosome 5 [del(5q)], with or without additional karyotypic abnormalities, is present in 10-15% of myelodysplastic syndromes (MDS). Anemia in these MDS responds less often to erythroblastic stimulating agents. However, immunomodulatory, anti-cytokine, and anti-angiogenic agent Lenalidomide (CC5013, Revlimid®) leads to red blood cells transfusion...

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Deregulation of innate immune signaling in myelodysplastic syndromes is associated with deletion of chromosome arm 5q.

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

Myelodysplastic syndrome (MDS) with interstitial deletion of a segment of the long arm of chromosome 5q [del(5q)] is characterized by bone marrow erythroid hyperplasia, atypical megakaryocytes, thrombocythemia, refractory anemia, and low risk of progression to acute myeloid leukemia (AML) compared with other types of MDS. The long arm of chromosome 5 contains two distinct commonly deleted regio...

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عنوان ژورنال:
  • Blood

دوره 124 5  شماره 

صفحات  -

تاریخ انتشار 2014