دورية أكاديمية
أعرض في EDS

Degraded speech sound processing in a rat model of fragile X syndrome.

التفاصيل البيبلوغرافية
العنوان: Degraded speech sound processing in a rat model of fragile X syndrome.
المؤلفون: Engineer CT; School of Behavioral and Brain Sciences, The University of Texas at Dallas, 800 West Campbell Road GR41, Richardson, TX 75080, United States. Electronic address: novitski@utdallas.edu., Centanni TM; School of Behavioral and Brain Sciences, The University of Texas at Dallas, 800 West Campbell Road GR41, Richardson, TX 75080, United States., Im KW; School of Behavioral and Brain Sciences, The University of Texas at Dallas, 800 West Campbell Road GR41, Richardson, TX 75080, United States., Rahebi KC; School of Behavioral and Brain Sciences, The University of Texas at Dallas, 800 West Campbell Road GR41, Richardson, TX 75080, United States., Buell EP; School of Behavioral and Brain Sciences, The University of Texas at Dallas, 800 West Campbell Road GR41, Richardson, TX 75080, United States., Kilgard MP; School of Behavioral and Brain Sciences, The University of Texas at Dallas, 800 West Campbell Road GR41, Richardson, TX 75080, United States.
المصدر: Brain research [Brain Res] 2014 May 20; Vol. 1564, pp. 72-84. Date of Electronic Publication: 2014 Apr 05.
نوع المنشور: Journal Article; Research Support, N.I.H., Extramural
اللغة: English
بيانات الدورية: Publisher: Elsevier/North-Holland Biomedical Press Country of Publication: Netherlands NLM ID: 0045503 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1872-6240 (Electronic) Linking ISSN: 00068993 NLM ISO Abbreviation: Brain Res Subsets: MEDLINE
أسماء مطبوعة: Original Publication: Amsterdam Elsevier/North-Holland Biomedical Press.
مواضيع طبية MeSH: Auditory Cortex/*physiopathology , Auditory Perception/*physiology , Discrimination, Psychological/*physiology , Fragile X Mental Retardation Protein/*genetics , Fragile X Syndrome/*physiopathology , Neurons/*physiology, Animals ; Disease Models, Animal ; Gene Knockout Techniques ; Male ; Phonetics ; Rats
مستخلص: Fragile X syndrome is the most common inherited form of intellectual disability and the leading genetic cause of autism. Impaired phonological processing in fragile X syndrome interferes with the development of language skills. Although auditory cortex responses are known to be abnormal in fragile X syndrome, it is not clear how these differences impact speech sound processing. This study provides the first evidence that the cortical representation of speech sounds is impaired in Fmr1 knockout rats, despite normal speech discrimination behavior. Evoked potentials and spiking activity in response to speech sounds, noise burst trains, and tones were significantly degraded in primary auditory cortex, anterior auditory field and the ventral auditory field. Neurometric analysis of speech evoked activity using a pattern classifier confirmed that activity in these fields contains significantly less information about speech sound identity in Fmr1 knockout rats compared to control rats. Responses were normal in the posterior auditory field, which is associated with sound localization. The greatest impairment was observed in the ventral auditory field, which is related to emotional regulation. Dysfunction in the ventral auditory field may contribute to poor emotional regulation in fragile X syndrome and may help explain the observation that later auditory evoked responses are more disturbed in fragile X syndrome compared to earlier responses. Rodent models of fragile X syndrome are likely to prove useful for understanding the biological basis of fragile X syndrome and for testing candidate therapies.
(Copyright © 2014 Elsevier B.V. All rights reserved.)
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معلومات مُعتمدة: R01 DC010433 United States DC NIDCD NIH HHS; 5R01DC010433 United States DC NIDCD NIH HHS
فهرسة مساهمة: Keywords: Auditory cortex; Fragile X syndrome; Speech
المشرفين على المادة: 0 (Fmr1 protein, rat)
139135-51-6 (Fragile X Mental Retardation Protein)
تواريخ الأحداث: Date Created: 20140410 Date Completed: 20141209 Latest Revision: 20211021
رمز التحديث: 20240628
مُعرف محوري في PubMed: PMC4034469
DOI: 10.1016/j.brainres.2014.03.049
PMID: 24713347
قاعدة البيانات: MEDLINE
الوصف
تدمد:1872-6240
DOI:10.1016/j.brainres.2014.03.049