title: Breathing abnormalities in a female mouse model of Rett syndrome.
abstract: Rett syndrome (RTT) is a female neurodevelopmental disease with breathing abnormalities. To understand whether breathing defects occur in the early lives of a group of female Mecp2(+/-) mice, a mouse model of RTT, and what percentage of mice shows RTT-like breathing abnormality, breathing activity was measured by plethysmography in conscious mice. Breathing frequency variation and central apnea in a group of Mecp2(+/-) females displayed a distribution pattern similar to Mecp2(-/Y) males, while the rest resembled the wild-type mice. Similar results were obtained using the k-mean clustering statistics analysis. With two independent methods, about 20% of female Mecp2(+/-) mice showed RTT-like breathing abnormalities that began as early as 3 weeks of age in the Mecp2(+/-) mice, and were suppressed with 3% CO2. The finding that only a small proportion of Mecp2(+/-) mice develops RTT-like breathing abnormalities suggests incomplete allele inactivation in the RTT-model Mecp2(+/-) mice.
authors.affiliation: Department of Biology, Georgia State University, 50 Decatur Street, Atlanta, GA, 30302, USA.
authors.forename: Christopher M
authors.initials: CM
authors.lastname: Johnson
authors.forename: Ningren
authors.initials: N
authors.lastname: Cui
authors.forename: Weiwei
authors.initials: W
authors.lastname: Zhong
authors.forename: Max F
authors.initials: MF
authors.lastname: Oginsky
authors.forename: Chun
authors.initials: C
authors.lastname: Jiang
mesh_terms: D000280:Administration, Inhalation; D000367:Age Factors; D000818:Animals; D001049:Apnea / Q000235:genetics / Q000503:physiopathology; D002245:Carbon Dioxide / Q000008:administration & dosage; D004195:Disease Models, Animal; D005260:Female; D020022:Genetic Predisposition to Disease; D006579:Heterozygote; D008168:Lung / Q000503:physiopathology*; D051783:Methyl-CpG-Binding Protein 2 / Q000172:deficiency / Q000235:genetics; D018345:Mice, Knockout; D010641:Phenotype; D010991:Plethysmography; D012119:Respiration; D056152:Respiratory Rate; D015518:Rett Syndrome / Q000235:genetics / Q000503:physiopathology* / Q000628:therapy
chemical_list: C497393:Mecp2 protein, mouse; D051783:Methyl-CpG-Binding Protein 2; D002245:Carbon Dioxide