Document Type

Article

Publication Date

12-1-2022

Publication Title

Sci Rep

Keywords

JMG, Mice, Animals, Mice, Knockout, Ciliopathies, Gene Knockout Techniques, Cilia, Databases, Factual, Nerve Tissue Proteins, Cell Cycle Proteins

Volume

12

Issue

1

First Page

20791

ISSN

2045-2322

PMID

36456625

DOI

https://doi.org/10.1038/s41598-022-19710-7

Grant

The authors thank all the various funding agencies supporting the International Mouse Phenotyping Consortium. The authors gratefully acknowledge their funding sources, including the Government of Canada through Genome Canada/Ontario Genomics OGI-051 (CM), NIH K08EY027463, NIH R03OD032622 (AM). NIH U54HG006364, U42OD011175, 5UM1OD02322, and UM1OD023321 (KCKL and CM). Hands-on mouse IMPC phenotyping has been carried out by a large number of laboratory staff with superb experimental skills and unsurpassed dedication.

Abstract

We searched a database of single-gene knockout (KO) mice produced by the International Mouse Phenotyping Consortium (IMPC) to identify candidate ciliopathy genes. We first screened for phenotypes in mouse lines with both ocular and renal or reproductive trait abnormalities. The STRING protein interaction tool was used to identify interactions between known cilia gene products and those encoded by the genes in individual knockout mouse strains in order to generate a list of "candidate ciliopathy genes." From this list, 32 genes encoded proteins predicted to interact with known ciliopathy proteins. Of these, 25 had no previously described roles in ciliary pathobiology. Histological and morphological evidence of phenotypes found in ciliopathies in knockout mouse lines are presented as examples (genes Abi2, Wdr62, Ap4e1, Dync1li1, and Prkab1). Phenotyping data and descriptions generated on IMPC mouse line are useful for mechanistic studies, target discovery, rare disease diagnosis, and preclinical therapeutic development trials. Here we demonstrate the effective use of the IMPC phenotype data to uncover genes with no previous role in ciliary biology, which may be clinically relevant for identification of novel disease genes implicated in ciliopathies.

Comments

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