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Studies in an Early Development Window Unveils a Severe HSC Defect in both Murine and Human Fanconi Anemia.

Abstract：

:Fanconi anemia (FA) causes bone marrow failure early during childhood, and recent studies indicate that a hematopoietic defect could begin in utero. We performed a unique kinetics study of hematopoiesis in Fancg-/- mouse embryos, between the early embryonic day 11.5 (E11.5) to E12.5 developmental window (when the highest level of hematopoietic stem cells [HSC] amplification takes place) and E14.5. This study reveals a deep HSC defect with exhaustion of proliferative and self-renewal capacities very early during development, together with severe FA clinical and biological manifestations, which are mitigated at E14.5 due to compensatory mechanisms that help to ensure survival of Fancg-/- embryos. It also reports that a deep HSC defect is also observed during human FA development, and that human FA fetal liver (FL) HSCs present a transcriptome profile similar to that of mouse E12.5 Fancg-/- FL HSCs. Altogether, our results highlight that early mouse FL could represent a good alternative model for studying Fanconi pathology.

journal_name

Stem Cell Reports

journal_title

Stem cell reports

authors

Domenech C,Maillard L,Rousseau A,Guidez F,Petit L,Pla M,Clay D,Guimiot F,Sanfilippo S,Jacques S,de la Grange P,Robil N,Soulier J,Souyri M

doi

10.1016/j.stemcr.2018.10.001

subject

Has Abstract

pub_date

2018-11-13 00:00:00

pages

1075-1091

issue

5

issn

2213-6711

pii

S2213-6711(18)30424-7

journal_volume

11

pub_type

杂志文章

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