Deregulated protein homeostasis constrains fetal hematopoietic stem cell pool expansion in Fanconi anemia
N Kovuru, M Mochizuki-Kashio, T Menna… - Nature …, 2024 - nature.com
Demand-adjusted and cell type specific rates of protein synthesis represent an important
safeguard for fate and function of long-term hematopoietic stem cells. Here, we identify …
safeguard for fate and function of long-term hematopoietic stem cells. Here, we identify …
RUNX1 mutations mitigate quiescence to promote transformation of hematopoietic progenitors in Fanconi anemia
W Marion, T Koppe, CC Chen, D Wang, K Frenis… - Leukemia, 2023 - nature.com
Many inherited bone marrow failure syndromes (IBMFSs) present a high risk of
transformation to myelodysplastic syndrome (MDS) and acute myeloid leukemia (AML) …
transformation to myelodysplastic syndrome (MDS) and acute myeloid leukemia (AML) …
Why hematopoietic stem cells fail in Fanconi anemia: Mechanisms and models
S Liu, ES Vivona, P Kurre - BioEssays, 2024 - Wiley Online Library
Fanconi anemia (FA) is generally classified as a DNA repair disorder, conferring a genetic
predisposition to cancer and prominent bone marrow failure (BMF) in early childhood …
predisposition to cancer and prominent bone marrow failure (BMF) in early childhood …
Loss of the homologous recombination gene rad51 leads to Fanconi anemia-like symptoms in zebrafish
JG Botthof, E Bielczyk-Maczyńska… - Proceedings of the …, 2017 - National Acad Sciences
RAD51 is an indispensable homologous recombination protein, necessary for strand
invasion and crossing over. It has recently been designated as a Fanconi anemia (FA) gene …
invasion and crossing over. It has recently been designated as a Fanconi anemia (FA) gene …
An induced pluripotent stem cell model of Fanconi anemia reveals mechanisms of p53-driven progenitor cell differentiation
W Marion, S Boettcher, S Ruiz-Torres… - Blood …, 2020 - ashpublications.org
Fanconi anemia (FA) is a disorder of DNA repair that manifests as bone marrow (BM) failure.
The lack of accurate murine models of FA has refocused efforts toward differentiation of …
The lack of accurate murine models of FA has refocused efforts toward differentiation of …
Abnormal migration behavior linked to Rac1 signaling contributes to primordial germ cell exhaustion in Fanconi anemia pathway-deficient Fancg−/− embryos
A Jarysta, L Riou, V Firlej, C Lapoujade… - Human Molecular …, 2022 - academic.oup.com
Fanconi anemia (FA) is a rare human genetic disorder characterized by bone marrow
failure, predisposition to cancer and developmental defects including hypogonadism …
failure, predisposition to cancer and developmental defects including hypogonadism …
Studies in an early development window unveils a severe HSC defect in both murine and human Fanconi anemia
C Domenech, L Maillard, A Rousseau, F Guidez, L Petit… - Stem cell reports, 2018 - cell.com
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 …
indicate that a hematopoietic defect could begin in utero. We performed a unique kinetics …
Replication stress increases mitochondrial metabolism and mitophagy in FANCD2 deficient fetal liver hematopoietic stem cells
M Mochizuki-Kashio, N Otsuki, K Fujiki… - Frontiers in …, 2023 - frontiersin.org
Fanconi Anemia (FA) is an inherited bone marrow (BM) failure disorder commonly
diagnosed during school age. However, in murine models, disrupted function of FA genes …
diagnosed during school age. However, in murine models, disrupted function of FA genes …
[HTML][HTML] Hematopoietic development: a gap in our understanding of inherited bone marrow failure
P Kurre - Experimental hematology, 2018 - Elsevier
Highlights•Inherited bone marrow failure syndromes reveal distinct hematopoietic defects
during development.•Murine, zebrafish. and induced pluripotent stem cell models have been …
during development.•Murine, zebrafish. and induced pluripotent stem cell models have been …
FANCD2 alleviates physiologic replication stress in fetal liver HSC
M Mochizuki-Kashio, Y me Yoon, T Menna, M Grompe… - Biorxiv, 2020 - biorxiv.org
Bone marrow failure (BMF) in Fanconi Anemia (FA) results from exhaustion of hematopoietic
stem cells (HSC), but the physiological role of FA proteins in HSC pool integrity remains …
stem cells (HSC), but the physiological role of FA proteins in HSC pool integrity remains …