@article{1f26d134e0dd4346a4ff60eb4e4f7a2a,
title = "Shank3-deficient rats exhibit degraded cortical responses to sound",
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.",
keywords = "22q13 deletion, Phelan-McDermid syndrome, SHANK3-haploinsufficiency syndromes, autism",
author = "Engineer, {Crystal T.} and Rahebi, {Kimiya C.} and Borland, {Michael S.} and Buell, {Elizabeth P.} and Im, {Kwok W.} and Wilson, {Linda G.} and Pryanka Sharma and Sven Vanneste and Hala Harony-Nicolas and Buxbaum, {Joseph D.} and Kilgard, {Michael P.}",
note = "Funding Information: We would like to thank Corey Lane, Nicole Moreno, and Meghan Pantalia for assistance with neural recordings. Ozlem Bozdagi and Seth Hays gave insightful suggestions on earlier versions of the manuscript. This research was supported by a grant from the National Institutes of Health to MPK (Grant # R01DC010433), the Seaver Foundation and a generous gift from William G. Gibson and Paulina Rychenkova. This research was also supported by a HeART grant from the International Rett Syndrome Foundation to MPK (Grant # 3206). This program was supported by the Defense Advanced Research Projects Agency (DARPA) Biological Technologies Office (BTO) Electrical Prescriptions (ElectRx) program under the auspices of Dr. Doug Weber through the Space and Naval Warfare Systems Center, Pacific Cooperative Agreement No. HR0011-15-2-0017 and N66001-15-2-4057 and the Targeted Neuroplasticity Training (TNT) program under the auspices of Dr. Doug Weber through the Space and Naval Warfare Systems Center, Pacific Grant/Contract No. N66001-17-2-4011. Funding Information: We would like to thank Corey Lane, Nicole Moreno, and Meghan Pantalia for assistance with neural recordings. Ozlem Bozdagi and Seth Hays gave insightful suggestions on earlier versions of the manuscript. This research was supported by a grant from the National Institutes of Health to MPK (Grant # R01DC010433), the Seaver Foundation and a generous gift from William G. Gibson and Paulina Rychenkova. This research was also supported by a HeART grant from the International Rett Syndrome Foundation to MPK (Grant # 3206). This program was supported by the Defense Advanced Research Projects Agency (DARPA) Biological Technologies Office (BTO) Electrical Prescriptions (ElectRx) program under the auspices of Dr. Doug Weber through the Space and Naval Warfare Systems Center, Pacific Cooperative Agreement No. HR0011-15-2-0017 and N66001-15-2-4057 and the Targeted Neuro-plasticity Training (TNT) program under the auspices of Dr. Doug Weber through the Space and Naval Warfare Systems Center, Pacific Grant/Contract No. N66001-17-2-4011. Publisher Copyright: {\textcopyright} 2017 International Society for Autism Research, Wiley Periodicals, Inc.",
year = "2018",
month = jan,
doi = "10.1002/aur.1883",
language = "English",
volume = "11",
pages = "59--68",
journal = "Autism Research",
issn = "1939-3792",
publisher = "John Wiley & Sons Inc.",
number = "1",
}