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Developmental dyslexia is a neurodevelopmental disorder that affects reading ability caused by genetic and non-genetic factors. Amongst the susceptibility genes identified to date, KIAA0319 is a prime candidate. RNA-interference experiments in rats suggested its involvement in cortical migration but we could not confirm these findings in Kiaa0319-mutant mice. Given its homologous gene Kiaa0319L (AU040320) has also been proposed to play a role in neuronal migration, we interrogated whether absence of AU040320 alone or together with KIAA0319 affects migration in the developing brain. Analyses of AU040320 and double Kiaa0319;AU040320 knockouts (dKO) revealed no evidence for impaired cortical lamination, neuronal migration, neurogenesis or other anatomical abnormalities. However, dKO mice displayed an auditory deficit in a behavioral gap-in-noise detection task. In addition, recordings of click-evoked auditory brainstem responses revealed suprathreshold deficits in wave III amplitude in AU040320-KO mice, and more general deficits in dKOs. These findings suggest that absence of AU040320 disrupts firing and/or synchrony of activity in the auditory brainstem, while loss of both proteins might affect both peripheral and central auditory function. Overall, these results stand against the proposed role of KIAA0319 and AU040320 in neuronal migration and outline their relationship with deficits in the auditory system.

Original publication

DOI

10.1093/cercor/bhx269

Type

Journal article

Journal

Cereb Cortex

Publication Date

01/12/2017

Volume

27

Pages

5831 - 5845

Keywords

auditory function, cerebral cortex, dyslexia, neuronal migration, temporal processing, Action Potentials, Adaptation, Physiological, Animals, Auditory Perception, Cell Movement, Cerebral Cortex, Dyslexia, Evoked Potentials, Auditory, Brain Stem, Female, Male, Mice, Inbred C57BL, Mice, Knockout, Nerve Tissue Proteins, Neurogenesis, Neurons, Receptors, Cell Surface