Mesenchymal stem/stromal cells from a transplanted, asymptomatic patient with Fanconi anemia exhibit an aging-like phenotype and dysregulated expression of genes implicated in hematopoiesis and myelodysplasia.

ABSTRACT:

Background aims

Fanconi anemia (FA) is an inherited bone marrow failure syndrome caused by defects in the repair of DNA inter-strand crosslinks and manifests as aplastic anemia, myelodysplastic syndrome and acute myeloid leukemia. FA also causes defects in mesenchymal stromal cell (MSC) function, but how different FA gene mutations alter function remains understudied.

Methods

We compared the growth, differentiation and transcript profile of a single MSC isolate from an asymptomatic patient with FA with a FANCG nonsense mutation who underwent hematopoietic stem cell transplantation 10 years prior to that from a representative healthy donor (HD).

Results

We show that FANCG^-/- MSCs exhibit rapid onset of growth cessation, skewed bi-lineage differentiation in f

SUBMITTER: Haga CL 

PROVIDER: S-EPMC10006355 | biostudies-literature | 2023 Apr

REPOSITORIES: biostudies-literature