Nephronophthisis gene products display RNA-binding properties and are recruited to stress granules

Mallarino, Luisa Estrada; Engel, Christina; Ilık, İbrahim Avşar; Maticzka, Daniel; Heyl, Florian; Müller, Barbara; Yakulov, Toma A.; Dengjel, Jörn; Backofen, Rolf; Akhtar, Asifa; Walz, Gerd

doi:10.1038/s41598-020-72905-8

Back

Journal article

+ 4 other files

Nephronophthisis gene products display RNA-binding properties and are recruited to stress granules

Mallarino, Luisa Estrada Renal Division, Department of Medicine, University Freiburg Medical Center, Faculty of Medicine, University of Freiburg, Hugstetter Str. 55, 79106 Freiburg, Germany - Faculty of Biology, University of Freiburg, Freiburg, Germany
Engel, Christina Renal Division, Department of Medicine, University Freiburg Medical Center, Faculty of Medicine, University of Freiburg, Hugstetter Str. 55, 79106 Freiburg, Germany
Ilık, İbrahim Avşar Max Planck Institute of Immunobiology and Epigenetics, Freiburg, Germany - Max Planck Institute for Molecular Genetics, 14195 Berlin, Germany
Maticzka, Daniel Institute for Informatics, Albert-Ludwigs-University, Freiburg, Germany
Heyl, Florian Institute for Informatics, Albert-Ludwigs-University, Freiburg, Germany
Müller, Barbara Renal Division, Department of Medicine, University Freiburg Medical Center, Faculty of Medicine, University of Freiburg, Hugstetter Str. 55, 79106 Freiburg, Germany
Yakulov, Toma A. Renal Division, Department of Medicine, University Freiburg Medical Center, Faculty of Medicine, University of Freiburg, Hugstetter Str. 55, 79106 Freiburg, Germany
Dengjel, Jörn Department of Dermatology, Medical Center, and Freiburg Institute for Advanced Studies, University of Freiburg, Freiburg, Germany - Department of Biology, University of Fribourg, 1700 Fribourg, Switzerland
Backofen, Rolf Institute for Informatics, Albert-Ludwigs-University, Freiburg, Germany
Akhtar, Asifa Max Planck Institute of Immunobiology and Epigenetics, Freiburg, Germany - CIBSS‑Centre for Integrative Biological Signalling Studies, University of Freiburg, Freiburg, Germany
Walz, Gerd Renal Division, Department of Medicine, University Freiburg Medical Center, Faculty of Medicine, University of Freiburg, Hugstetter Str. 55, 79106 Freiburg, Germany - Signalling Research Centres BIOSS and CIBSS, University of Freiburg, Freiburg, Germany

29.09.2020

Published in:

Scientific Reports. - 2020, vol. 10, no. 1, p. 15954

English Mutations of cilia-associated molecules cause multiple developmental defects that are collectively termed ciliopathies. However, several ciliary proteins, involved in gating access to the cilium, also assume localizations at other cellular sites including the nucleus, where they participate in DNA damage responses to maintain tissue integrity. Molecular insight into how these molecules execute such diverse functions remains limited. A mass spectrometry screen for ANKS6-interacting proteins suggested an involvement of ANKS6 in RNA processing and/or binding. Comparing the RNA- binding properties of the known RNA-binding protein BICC1 with the three ankyrin- repeat proteins ANKS3, ANKS6 (NPHP16) and INVERSIN (NPHP2) confirmed that certain nephronophthisis (NPH) family members can interact with RNA molecules. We also observed that BICC1 and INVERSIN associate with stress granules in response to translational inhibition. Furthermore, BICC1 recruits ANKS3 and ANKS6 into TIA-1- positive stress granules after exposure to hippuristanol. Our findings uncover a novel function of NPH family members, and provide further evidence that NPH family members together with BICC1 are involved in stress responses to maintain tissue and organ integrity.

Faculty

Faculté des sciences et de médecine

Department

Département de Biologie

Language