Mutated neuron navigator 3 as a candidate gene for a rare neurodevelopmental disorder

Document Type

Article

Department

Pathology and Laboratory Medicine

Abstract

Background: Neuron navigator 3 (NAV3) is characterized as one of the neuron navigator family (NAV1, NAV2, NAV3) proteins predominantly expressed in the nervous system. The NAV3-encoded protein comprises a conserved AAA and coiled-coil domains characteristic of ATPases, which are associated with different cellular activities.
Methods: We describe a Saudi proband presenting a complex recessive neurodevelopmental disorder (NDD). Whole exome sequencing (WES) followed by Sanger sequencing, 3D protein modeling and RT-qPCR was performed.
Results: WES revealed a bi-allelic frameshift variant (c.2604_2605delAG; p.Val870SerfsTer12) in exon 12 of the NAV3 gene. Furthermore, RT-qPCR revealed a significant decrease in the NAV3 mRNA expression in the patient sample, and 3D protein modeling revealed disruption of the overall secondary structure.
Conclusion: For the time, we associate a bi-allelic variant in the NAV3 gene causing NDD in humans.

Comments

Pagination are not provided by the author/publisher.

Publication (Name of Journal)

Molecular Genetics & Genomic Medicine

DOI

10.1002/mgg3.2473

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