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Autism Res. 2018 Jan;11(1):59-68. doi: 10.1002/aur.1883. Epub 2017 Oct 20.

Shank3-deficient rats exhibit degraded cortical responses to sound.

Author information

1
School of Behavioral and Brain Sciences, The University of Texas at Dallas, 800 West Campbell Road BSB11, Richardson, TX, 75080.
2
Texas Biomedical Device Center, The University of Texas at Dallas, 800 West Campbell Road BSB11, Richardson, TX, 75080.
3
Seaver Autism Center for Research and Treatment, Icahn School of Medicine at Mount Sinai, New York, NY.
4
Department of Psychiatry, Icahn School of Medicine at Mount Sinai, New York, NY.
5
Friedman Brain Institute, Icahn School of Medicine at Mount Sinai, New York, NY.
6
Fishberg Department of Neuroscience, Icahn School of Medicine at Mount Sinai, New York, NY.
7
Department of Genetics and Genomic Sciences, Icahn School of Medicine at Mount Sinai, New York, NY.
8
The Mindich Child Health and Development Institute, Icahn School of Medicine at Mount Sinai, New York, NY.

Abstract

Individuals with SHANK3 mutations have severely impaired receptive and expressive language abilities. While brain responses are known to be abnormal in these individuals, the auditory cortex response to sound has remained largely understudied. In this study, we document the auditory cortex response to speech and non-speech sounds in the novel Shank3-deficient rat model. We predicted that the auditory cortex response to sounds would be impaired in Shank3-deficient rats. We found that auditory cortex responses were weaker in Shank3 heterozygous rats compared to wild-type rats. Additionally, Shank3 heterozygous responses had less spontaneous auditory cortex firing and were unable to respond well to rapid trains of noise bursts. The rat model of the auditory impairments in SHANK3 mutation could be used to test potential rehabilitation or drug therapies to improve the communication impairments observed in individuals with Phelan-McDermid syndrome. Autism Res 2018, 11: 59-68. © 2017 International Society for Autism Research, Wiley Periodicals, Inc.

LAY SUMMARY:

Individuals with SHANK3 mutations have severely impaired language abilities, yet the auditory cortex response to sound has remained largely understudied. In this study, we found that auditory cortex responses were weaker and were unable to respond well to rapid sounds in Shank3-deficient rats compared to control rats. The rat model of the auditory impairments in SHANK3 mutation could be used to test potential rehabilitation or drug therapies to improve the communication impairments observed in individuals with Phelan-McDermid syndrome.

KEYWORDS:

22q13 deletion; Phelan-McDermid syndrome; SHANK3-haploinsufficiency syndromes; autism

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