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Studies in an early development window unveils a severe HSC defect in both murine and human Fanconi anemia

ABSTRACT: Studies in an early development window unveils a severe HSC defect in both murine and human Fanconi anemia

PROVIDER: PRJNA491828 | ENA |

REPOSITORIES: ENA

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Studies in an early development window unveils a severe HSC defect in both murine and human Fanconi anemia [human]

Project description:We performed a unique kinetics study of hematopoiesis in Fancg-/- mouse embryos, between E12.5 and E14.5 developmental window. We also report for the first time that a deep HSC defect is also observed during human FA development, and that human FA FL HSCs present a transcriptome profile similar to that of mouse E12.5 Fancg-/- FL HSCs.

2018-11-30 | GSE120168 | GEO

Studies in an early development window unveils a severe HSC defect in both murine and human Fanconi anemia [mouse]

2018-11-30 | GSE120167 | GEO

Studies in an early development window unveils a severe HSC defect in both murine and human Fanconi anemia [mouse]

Project description:Studies in an early development window unveils a severe HSC defect in both murine and human Fanconi anemia [mouse]

| PRJNA491829 | ENA

Studies in an early development window unveils a severe HSC defect in both murine and human Fanconi anemia [human]

Project description:Studies in an early development window unveils a severe HSC defect in both murine and human Fanconi anemia [human]

| PRJNA491830 | ENA

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

Project description: Not available

| S-EPMC6234961 | biostudies-literature

Studies in an early development window unveils a severe HSC defect in both murine and human Fanconi anemia

Project description:This SuperSeries is composed of the SubSeries listed below.

2018-11-30 | GSE120169 | GEO

Modeling clonal evolution in Fanconi anemia

Project description:Transcriptomic comparison of stages of clonal evolution of Fanconi anemia modeled using induced pluripotent stem cells.

2024-04-17 | GSE205844 | GEO

BM clonal evolution in Fanconi anemia patients

Project description:Arrays-CGH and/or SNP-array data from n=57 Fanconi anemia BM and n=36 paired fibroblasts

2011-07-28 | E-MTAB-529 | biostudies-arrayexpress

Fanconi

Project description:Fanconi

| PRJEB5057 | ENA

Pluripotent cell models of Fanconi anemia identify the early pathological defect in human hemoangiogenic progenitors

Project description:Fanconi anemia (FA) is a disorder of genomic instability characterized by progressive bone marrow failure (BMF), developmental abnormalities and an increased susceptibility to cancer. Although various consequences in hematopoietic stem/progenitor cells have been attributed to FA-BMF, the quest to identify the initial pathological event is still ongoing. To address this issue, we established induced pluripotent stem cells (iPSCs) from fibroblasts of six FA patients with FANCA mutations. An improved reprogramming method yielded iPSC-like colonies from all patients, and iPSC clones were propagated from two patients. Quantitative evaluation of the differentiation ability demonstrated that the differentiation propensity toward the hematopoietic and endothelial lineages is already defective in early hemoangiogenic progenitors. The expression levels of critical transcription factors were significantly downregulated in these progenitors. These data indicate that the hematopoietic consequences in FA patients originate from the early hematopoietic stage, and highlight the potential usefulness of iPSC technology for elucidating the pathogenesis of FA-BMF. The investigation of the RNA-seq analysis of iPSC-derived HAPCs.

2015-02-03 | E-GEOD-65507 | biostudies-arrayexpress

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