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NME7  (non-metastatic cells 7, nucleoside diphosphate kinase 7) is a member of a gene family with a profound effect on health/disease status. NME7 is an established member of the ciliome and contributes to the regulation of the microtubule-organizing center. We aimed to create a rat model to further investigate the phenotypic consequences of  Nme7  gene deletion. The CRISPR/Cas9 nuclease system was used for the generation of Sprague Dawley  Nme7  knock-out rats targeting the exon 4 of the  Nme7  gene. We found the homozygous  Nme7  gene deletion to be semi-lethal, as the majority of SD  Nme7   −/−  pups died prior to weaning. The most prominent phenotypes in surviving SD  Nme7   −/−  animals were hydrocephalus, situs inversus totalis, postnatal growth retardation, and sterility of both sexes. Thinning of the neocortex was histologically evident at 13.5 day of gestation, dilation of all ventricles was detected at birth, and an external sign of hydrocephalus, i.e., doming of the skull, was usually apparent at 2 weeks of age. Heterozygous SD  Nme7   +/−  rats developed normally; we did not detect any symptoms of primary ciliary dyskinesia. The transcriptomic profile of liver and lungs corroborated the histological findings, revealing defects in cell function and viability. In summary, the knock-out of the rat  Nme7  gene resulted in a range of conditions consistent with the presentation of primary ciliary dyskinesia, supporting the previously implicated role of the centrosomally located  Nme7  gene in ciliogenesis and control of ciliary transport.

Semi-Lethal Primary Ciliary Dyskinesia in Rats Lacking the Nme7 Gene