Studies in an Early Development Window Unveils a Severe HSC Defect in both Murine and Human Fanconi Anemia. 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. Stem Cell Reports, 11(5):1075–1091, November, 2018. doi abstract bibtex 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.
@article{domenech_studies_2018,
title = {Studies in an {Early} {Development} {Window} {Unveils} a {Severe} {HSC} {Defect} in both {Murine} and {Human} {Fanconi} {Anemia}},
volume = {11},
issn = {2213-6711},
doi = {10.1016/j.stemcr.2018.10.001},
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.},
language = {eng},
number = {5},
journal = {Stem Cell Reports},
author = {Domenech, Carine and Maillard, Loïc and Rousseau, Alix and Guidez, Fabien and Petit, Laurence and Pla, Marika and Clay, Denis and Guimiot, Fabien and Sanfilippo, Sandra and Jacques, Sebastien and de la Grange, Pierre and Robil, Noémie and Soulier, Jean and Souyri, Michèle},
month = nov,
year = {2018},
pmid = {30449320},
pmcid = {PMC6234961},
keywords = {Fanconi anemia, HSC, fetal liver, human embryonic development, mouse embryonic development, placenta, transcriptome},
pages = {1075--1091},
}
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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. 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