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Mutant ASXL1 induces age-related expansion of phenotypic hematopoietic stem cells through activation of Akt/mTOR pathway

UID: 10618

Publisher(s): The Institute of Medical Science

Description
Summary from BioProject: "Somatic mutations of ASXL1 are frequently detected in age-related clonal hematopoiesis (CH). However, how ASXL1 mutations drive CH remains elusive. Using knockin (KI) mice expressing a C-terminally truncated form of ASXL1-mutant (ASXL1-MT), we examined the influence of ASXL1-MT on physiological aging in hematopoietic stem cells (HSCs). HSCs expressing ASXL1-MT display competitive disadvantage after transplantation. Nevertheless, in genetic mosaic mouse model, they acquire clonal advantage during aging, recapitulating CH in humans. Mechanistically, ASXL1-MT cooperates with BAP1 to deubiquitinate and activate AKT. Overactive Akt/mTOR signaling induced by ASXL1-MT results in aberrant proliferation and dysfunction of HSCs associated with age-related accumulation of DNA damage. Treatment with an mTOR inhibitor rapamycin ameliorates aberrant expansion of the HSC compartment as well as dysregulated hematopoiesis in aged ASXL1-MT KI mice. Our findings suggest that ASXL1-MT provokes dysfunction of HSCs, whereas it confers clonal advantage on HSCs over time, leading to the development of CH."
Subject of Study
Subject(s)
Access via BioProject

Information about the experiment with access to sample data
Accession #: PRJNA673672

Access Restrictions
Free to All
Access Instructions
The BioProject and SRA databases provide open access to these files.
Associated Publications
Data Type
Equipment Used
Illumina HiSeq 2500
Dataset Format(s)
SRA
Data Tool(s)
RNA Seq
Dataset Size
38.9 GB (SRA)
Data Catalog Record Updated
2021-06-23