Zfrp8 forms a complex with fragile-X mental retardation protein and regulates its localization and function

Developmental Biology
William TanRuth Steward

Abstract

Fragile-X syndrome is the most commonly inherited cause of autism and mental disabilities. The Fmr1 (Fragile-X Mental Retardation 1) gene is essential in humans and Drosophila for the maintenance of neural stem cells, and Fmr1 loss results in neurological and reproductive developmental defects in humans and flies. FMRP (Fragile-X Mental Retardation Protein) is a nucleo-cytoplasmic shuttling protein, involved in mRNA silencing and translational repression. Both Zfrp8 and Fmr1 have essential functions in the Drosophila ovary. In this study, we identified FMRP, Nufip (Nuclear Fragile-X Mental Retardation Protein-interacting Protein) and Tral (Trailer Hitch) as components of a Zfrp8 protein complex. We show that Zfrp8 is required in the nucleus, and controls localization of FMRP in the cytoplasm. In addition, we demonstrate that Zfrp8 genetically interacts with Fmr1 and tral in an antagonistic manner. Zfrp8 and FMRP both control heterochromatin packaging, also in opposite ways. We propose that Zfrp8 functions as a chaperone, controlling protein complexes involved in RNA processing in the nucleus.

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Citations

Jun 12, 2019·Molecular and Cellular Biology·Joshua J BlackArlen W Johnson
Jun 12, 2020·The FEBS Journal·Mohamed S TahaMohammad R Ahmadian
Jul 23, 2020·Stem Cells International·Zhenzhen HanWei Li
May 17, 2017·International Journal of Molecular Sciences·Valeria SpecchiaMaria Pia Bozzetti
Mar 31, 2018·Frontiers in Molecular Neuroscience·Regina Dahlhaus

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