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Disease genetics

Corrupted USB1 fails to process microRNAs required for blood development

Nature Reviews Genetics volume 24, page 272 (2023)Cite this article

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Mutations in USB1 cause poikiloderma with neutropenia (PN) — a rare genetic disorder with defects in blood development — although the exact mechanisms were unknown. A new study in Science reports that USB1 removes destabilizing adenosines from microRNAs (miRNAs) that are essential for blood cell formation, which, if not removed, results in miRNA degradation and the clinical features of PN.

USB1 is known to process U6 and U6atac small nuclear RNAs (snRNAs). However, levels of these snRNAs were unaffected by loss of USB1, which indicates that other USB1 targets might be important. To identify these targets, the authors performed RNA sequencing in wild-type and USB1-mutant cells. Loss of USB1 caused a reduction in the abundance of several mRNAs known to be involved in neutrophil formation and haematopoiesis. Moreover, miRNAs involved in blood formation were reduced in abundance, and this could be rescued by expression of the wild-type USB1 protein in mutant cells. Thus, USB1 might regulate haematopoietic development by controlling miRNA homeostasis.

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References

Original article

  • Jeong, H.-C. et al. USB1 is a miRNA deadenylase that regulates hematopoietic development. Science 379, 901–907 (2023)

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  1. Nature Reviews Genetics http://www.nature.com/nrg/

    Michael Attwaters

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  1. Michael Attwaters
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Correspondence to Michael Attwaters.

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Attwaters, M. Corrupted USB1 fails to process microRNAs required for blood development. Nat Rev Genet 24, 272 (2023). https://doi.org/10.1038/s41576-023-00595-9

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  • DOI: https://doi.org/10.1038/s41576-023-00595-9

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