PuSH - Publication Server of Helmholtz Zentrum München: PTBP1 promotes hematopoietic stem cell maintenance and red blood cell development by ensuring sufficient availability of ribosomal constituents.

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Rehn, M.* ; Wenzel, A.* ; Frank, A.K.* ; Schuster, M.B.* ; Pundhir, S.* ; Jørgensen, N.* ; Vitting-Seerup, K.* ; Ge, Y.* ; Jendholm, J.* ; Michaut, M.* ; Schoof, E.M.* ; Jensen, T.L.* ; Rapin, N.* ; Sapio, R.T.* ; Andersen, K.L.* ; Lund, A.H.* ; Solimena, M. ; Holzenberger, M.* ; Pestov, D.G.* ; Porse, B.T.*

PTBP1 promotes hematopoietic stem cell maintenance and red blood cell development by ensuring sufficient availability of ribosomal constituents.

Cell Rep. 39:110793 (2022)

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Ribosomopathies constitute a range of disorders associated with defective protein synthesis mainly affecting hematopoietic stem cells (HSCs) and erythroid development. Here, we demonstrate that deletion of poly-pyrimidine-tract-binding protein 1 (PTBP1) in the hematopoietic compartment leads to the development of a ribosomopathy-like condition. Specifically, loss of PTBP1 is associated with decreases in HSC self-renewal, erythroid differentiation, and protein synthesis. Consistent with its function as a splicing regulator, PTBP1 deficiency results in splicing defects in hundreds of genes, and we demonstrate that the up-regulation of a specific isoform of CDC42 partly mimics the protein-synthesis defect associated with loss of PTBP1. Furthermore, PTBP1 deficiency is associated with a marked defect in ribosome biogenesis and a selective reduction in the translation of mRNAs encoding ribosomal proteins. Collectively, this work identifies PTBP1 as a key integrator of ribosomal functions and highlights the broad functional repertoire of RNA-binding proteins.

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