Motor, Somatosensory, Viscerosensory and Metabolic Impairments in a Heterozygous Female Rat Model of Rett Syndrome.

Autor: Bhattacherjee A; Department of Molecular and Integrative Physiology, University of Kansas Medical Center, Kansas City, KS 66160, USA. Aritra.Bhattacherjee@childrens.harvard.edu.; Kansas Intellectual and Developmental Disabilities Research Center, University of Kansas Medical Center, Kansas City, KS 66160, USA. Aritra.Bhattacherjee@childrens.harvard.edu., Winter MK; Kansas Intellectual and Developmental Disabilities Research Center, University of Kansas Medical Center, Kansas City, KS 66160, USA. mwinter2@kumc.edu., Eggimann LS; Kansas Intellectual and Developmental Disabilities Research Center, University of Kansas Medical Center, Kansas City, KS 66160, USA. leggimann@kumc.edu., Mu Y; Department of Molecular and Integrative Physiology, University of Kansas Medical Center, Kansas City, KS 66160, USA. mu2@uthsc.edu.; Kansas Intellectual and Developmental Disabilities Research Center, University of Kansas Medical Center, Kansas City, KS 66160, USA. mu2@uthsc.edu., Gunewardena S; Department of Molecular and Integrative Physiology, University of Kansas Medical Center, Kansas City, KS 66160, USA. sgunewardena@kumc.edu.; Kansas Intellectual and Developmental Disabilities Research Center, University of Kansas Medical Center, Kansas City, KS 66160, USA. sgunewardena@kumc.edu., Liao Z; Department of Molecular and Integrative Physiology, University of Kansas Medical Center, Kansas City, KS 66160, USA. zliao@kumc.edu.; Kansas Intellectual and Developmental Disabilities Research Center, University of Kansas Medical Center, Kansas City, KS 66160, USA. zliao@kumc.edu., Christianson JA; Kansas Intellectual and Developmental Disabilities Research Center, University of Kansas Medical Center, Kansas City, KS 66160, USA. jchristianson@kumc.edu.; Department of Anatomy and Cell Biology, University of Kansas Medical Center, Kansas City, KS 66160, USA. jchristianson@kumc.edu., Smith PG; Department of Molecular and Integrative Physiology, University of Kansas Medical Center, Kansas City, KS 66160, USA. psmith@kumc.edu.; Kansas Intellectual and Developmental Disabilities Research Center, University of Kansas Medical Center, Kansas City, KS 66160, USA. psmith@kumc.edu.
Jazyk: angličtina
Zdroj: International journal of molecular sciences [Int J Mol Sci] 2017 Dec 29; Vol. 19 (1). Date of Electronic Publication: 2017 Dec 29.
DOI: 10.3390/ijms19010097
Abstrakt: Rett Syndrome (RTT), an autism-related disorder caused by mutation of the X-linked Methyl CpG-binding Protein 2 ( MECP2 ) gene, is characterized by severe cognitive and intellectual deficits. While cognitive deficits are well-documented in humans and rodent models, impairments of sensory, motor and metabolic functions also occur but remain poorly understood. To better understand non-cognitive deficits in RTT, we studied female rats heterozygous for Mecp2 mutation ( Mecp2 -/x ); unlike commonly used male Mecp2 -/y rodent models, this more closely approximates human RTT where males rarely survive. Mecp2 -/x rats showed rapid, progressive decline of motor coordination through six months of age as assessed by rotarod performance, accompanied by deficits in gait and posture. Mecp2 -/x rats were hyper-responsive to noxious pressure and cold, but showed visceral hyposensitivity when tested by colorectal distension. Mecp2 -/x rats ate less, drank more, and had more body fat resulting in increased weight gain. Our findings reveal an array of progressive non-cognitive deficits in this rat model that are likely to contribute to the compromised quality of life that characterizes RTT.
Competing Interests: The authors declare no conflict of interest.
Databáze: MEDLINE
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