In the last few years, the advent of new technological approaches has led to a better knowledge of the ontogeny of erythropoiesis during development and of the journey leading from hematopoietic stem cells (HSCs) to mature red blood cells (RBCs). Our view of a well-defined hierarchical model of hematopoiesis with a near-homogeneous HSC population residing at the apex has been progressively challenged in favor of a landscape where HSCs themselves are highly heterogeneous and lineages separate earlier than previously thought. The coordination of these events is orchestrated by transcription factors (TFs) that work in a combinatorial manner to activate and/or repress their target genes. The development of next generation sequencing (NGS) has facilitated the identification of pathological mutations involving TFs underlying hematological defects. The examples of GATA1 and KLF1 presented in this review suggest that in the next few years the number of TF mutations associated with dyserythropoietic disorders will further increase.

Barbarani, G., Fugazza, C., Strouboulis, J., Ronchi, A. (2019). The pleiotropic effects of GATA1 and KLF1 in physiological erythropoiesis and in dyserythropoietic disorders. FRONTIERS IN PHYSIOLOGY, 10(FEB), 91 [10.3389/fphys.2019.00091].

The pleiotropic effects of GATA1 and KLF1 in physiological erythropoiesis and in dyserythropoietic disorders

Barbarani G.;Ronchi A. E.
2019

Abstract

In the last few years, the advent of new technological approaches has led to a better knowledge of the ontogeny of erythropoiesis during development and of the journey leading from hematopoietic stem cells (HSCs) to mature red blood cells (RBCs). Our view of a well-defined hierarchical model of hematopoiesis with a near-homogeneous HSC population residing at the apex has been progressively challenged in favor of a landscape where HSCs themselves are highly heterogeneous and lineages separate earlier than previously thought. The coordination of these events is orchestrated by transcription factors (TFs) that work in a combinatorial manner to activate and/or repress their target genes. The development of next generation sequencing (NGS) has facilitated the identification of pathological mutations involving TFs underlying hematological defects. The examples of GATA1 and KLF1 presented in this review suggest that in the next few years the number of TF mutations associated with dyserythropoietic disorders will further increase.
Articolo in rivista - Review Essay
Dyserythropoiesis; Erythropoiesis; GATA1; KLF1; Transcription factors
English
2019
10
FEB
91
91
partially_open
Barbarani, G., Fugazza, C., Strouboulis, J., Ronchi, A. (2019). The pleiotropic effects of GATA1 and KLF1 in physiological erythropoiesis and in dyserythropoietic disorders. FRONTIERS IN PHYSIOLOGY, 10(FEB), 91 [10.3389/fphys.2019.00091].
File in questo prodotto:
File Dimensione Formato  
10281-255729.pdf

accesso aperto

Tipologia di allegato: Publisher’s Version (Version of Record, VoR)
Dimensione 1.94 MB
Formato Adobe PDF
1.94 MB Adobe PDF Visualizza/Apri
Barbarani review J Physiology.pdf

Solo gestori archivio

Tipologia di allegato: Publisher’s Version (Version of Record, VoR)
Dimensione 1.94 MB
Formato Adobe PDF
1.94 MB Adobe PDF   Visualizza/Apri   Richiedi una copia

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/10281/255729
Citazioni
  • Scopus 16
  • ???jsp.display-item.citation.isi??? 13
Social impact