| Infantile spasms and Down syndrome: a new animal model. | |
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MedLine Citation:
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PMID: 19190545 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Infantile spasms is a catastrophic childhood seizure disorder for which few animal models exist. Children with Down syndrome are highly susceptible to infantile spasms. The Ts65Dn mouse is a valid model for Down syndrome; therefore, we tested the hypothesis that the Ts65Dn mouse represents a substrate for an animal model of infantile spasms. The baseline of naïve Ts65Dn mice showed spontaneous spike-and-wave discharges, a pattern that worsened with baclofen and gamma-butyrolactone, which induced acute epileptic extensor spasms (AEES) associated with epileptiform polyspike bursts and an electrodecremental response on the EEG. GABABR-agonist-induced AEES were significantly reduced with vigabatrin, rodent ACTH fragment, valproic acid, ethosuximide, and CGP 35348. Porcine ACTH had no effect. GABABR protein expression was significantly increased in the thalamus and medulla oblongata of Ts65D mice in comparison with wild-type controls. The GABABR agonist-treated Ts65Dn mouse shows the unique clinical, electrographic, and pharmacologic signature of infantile spasms and represents a valid, acute model of this disorder. GABABR-mediated mechanisms may contribute to the increased susceptibility of children with Down syndrome to infantile spasms. |
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Authors:
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Miguel A Cortez; Liqing Shen; Ying Wu; Ilyas S Aleem; Catherine H Trepanier; Hamid R Sadeghnia; Asim Ashraf; Ashlin Kanawaty; Chen-Chu Liu; Lee Stewart; O Carter Snead |
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Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't |
Journal Detail:
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Title: Pediatric research Volume: 65 ISSN: 1530-0447 ISO Abbreviation: Pediatr. Res. Publication Date: 2009 May |
Date Detail:
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Created Date: 2009-07-21 Completed Date: 2009-09-24 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 0100714 Medline TA: Pediatr Res Country: United States |
Other Details:
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Languages: eng Pagination: 499-503 Citation Subset: IM |
Affiliation:
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Division of Neurology, Hospital for Sick Children, Toronto, Ontario, Canada M5G 1X8. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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4-Butyrolactone* Animals Anticonvulsants / pharmacology Baclofen* Brain / drug effects, metabolism*, physiopathology Disease Models, Animal Down Syndrome / complications*, drug therapy, genetics, physiopathology Electroencephalography Ethosuximide / pharmacology GABA Agonists* Genotype Humans Infant Mice Mice, Mutant Strains Organophosphorus Compounds / pharmacology Phenotype Receptors, GABA-A / drug effects, metabolism Receptors, GABA-B / drug effects, metabolism* Spasms, Infantile / chemically induced*, genetics, physiopathology, prevention & control Time Factors Valproic Acid / pharmacology Vigabatrin / pharmacology |
| Chemical | |
Reg. No./Substance:
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0/Anticonvulsants; 0/GABA Agonists; 0/Organophosphorus Compounds; 0/Receptors, GABA-A; 0/Receptors, GABA-B; 1134-47-0/Baclofen; 123690-79-9/CGP 35348; 60643-86-9/Vigabatrin; 77-67-8/Ethosuximide; 96-48-0/4-Butyrolactone; 99-66-1/Valproic Acid |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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