Microphthalmia transcription factor expression contributes to bone marrow failure in Fanconi anemia

A Oppezzo, J Bourseguin, E Renaud… - The Journal of …, 2020 - Am Soc Clin Investig
Hematopoietic stem cell (HSC) attrition is considered the key event underlying progressive
BM failure (BMF) in Fanconi anemia (FA), the most frequent inherited BMF disorder in …

[HTML][HTML] MYC promotes bone marrow stem cell dysfunction in Fanconi anemia

A Rodríguez, K Zhang, A Färkkilä, J Filiatrault, C Yang… - Cell stem cell, 2021 - cell.com
Bone marrow failure (BMF) in Fanconi anemia (FA) patients results from dysfunctional
hematopoietic stem and progenitor cells (HSPCs). To identify determinants of BMF, we …

Aberrant activation of stress-response pathways leads to TNF-α oversecretion in Fanconi anemia

D Briot, G Macé-Aimé, F Subra… - Blood, The Journal of …, 2008 - ashpublications.org
Fanconi anemia (FA), an inherited syndrome that associates bone marrow failure, cancer
predisposition, and genetic instability, is characterized by an overproduction of the …

TGF-β pathway inhibition signals new hope for Fanconi anemia

H Tummala, I Dokal - Cell Stem Cell, 2016 - cell.com
An improved understanding of the bone marrow failure (BMF) mechanisms in Fanconi
anemia (FA) may improve current therapeutic strategies. Zhang et al. identify hyperactive …

[HTML][HTML] Bone Marrow Failure in Fanconi Anemia from Hyperactive TGF-β Signaling

H Zhang, D Kozono, K O'Connor, S Vidal-Cardenas… - Blood, 2014 - Elsevier
Fanconi anemia (FA) is the most common inherited bone marrow failure syndrome. FA
patients develop bone marrow failure during the first decade of life due to attrition of …

Disrupted signaling through the Fanconi anemia pathway leads to dysfunctional hematopoietic stem cell biology: underlying mechanisms and potential therapeutic …

A Geiselhart, A Lier, D Walter, MD Milsom - Anemia, 2012 - Wiley Online Library
Fanconi anemia (FA) is the most common inherited bone marrow failure syndrome. FA
patients suffer to varying degrees from a heterogeneous range of developmental defects …

TGF-β inhibition rescues hematopoietic stem cell defects and bone marrow failure in Fanconi anemia

H Zhang, DE Kozono, KW O'Connor, S Vidal-Cardenas… - Cell stem cell, 2016 - cell.com
Fanconi anemia (FA) is an inherited DNA repair disorder characterized by progressive bone
marrow failure (BMF) from hematopoietic stem and progenitor cell (HSPC) attrition. A greater …

Genetic disruption of both Fancc and Fancg in mice recapitulates the hematopoietic manifestations of Fanconi anemia

AC Pulliam-Leath, SL Ciccone… - Blood, The Journal …, 2010 - ashpublications.org
Fanconi anemia (FA) is an inherited chromosomal instability syndrome characterized by
bone marrow failure, myelodysplasia (MDS), and acute myeloid leukemia (AML). Eight FA …

The Fanconi anemia pathway is required for efficient repair of stress-induced DNA damage in haematopoietic stem cells

P Kaschutnig, R Bogeska, D Walter, A Lier… - Cell Cycle, 2015 - Taylor & Francis
Within regenerating tissues, aging is characterized by a progressive general deterioration of
organ function, thought to be driven by the gradual depletion of functional adult stem cells …

Endogenous DNA damage leads to p53-independent deficits in replicative fitness in fetal murine Fancd2−/− hematopoietic stem and progenitor cells

Y me Yoon, KJ Storm, AN Kamimae-Lanning… - Stem Cell Reports, 2016 - cell.com
Our mechanistic understanding of Fanconi anemia (FA) pathway function in hematopoietic
stem and progenitor cells (HSPCs) owes much to their role in experimentally induced DNA …