In:
PLOS ONE, Public Library of Science (PLoS), Vol. 16, No. 10 ( 2021-10-21), p. e0258830-
Abstract:
Rett syndrome (RTT) is a neurodevelopmental disorder with X-linked dominant inheritance caused mainly by mutations in the methyl-CpG-binding protein 2 ( MECP2 ) gene. The effects of various Mecp2 mutations have been extensively assessed in mouse models, but none adequately mimic the symptoms and pathological changes of RTT. In this study, we assessed the effects of Mecp2 gene deletion on female rats ( Mecp2 +/− ) and found severe impairments in social behavior [at 8 weeks (w), 12 w, and 23 w of age], motor function [at 16 w and 26 w] , and spatial cognition [at 29 w] as well as lower plasma insulin-like growth factor (but not brain-derived neurotrophic factor) and markedly reduced acetylcholine (30%–50%) in multiple brain regions compared to female Mecp2 +/+ rats [at 29 w]. Alternatively, changes in brain monoamine levels were relatively small, in contrast to reports on mouse Mecp2 mutants. Female Mecp2 -deficient rats express phenotypes resembling RTT and so may provide a robust model for future research on RTT pathobiology and treatment.
Type of Medium:
Online Resource
ISSN:
1932-6203
DOI:
10.1371/journal.pone.0258830
DOI:
10.1371/journal.pone.0258830.g001
DOI:
10.1371/journal.pone.0258830.g002
DOI:
10.1371/journal.pone.0258830.g003
DOI:
10.1371/journal.pone.0258830.g004
DOI:
10.1371/journal.pone.0258830.g005
DOI:
10.1371/journal.pone.0258830.g006
DOI:
10.1371/journal.pone.0258830.g007
DOI:
10.1371/journal.pone.0258830.g008
DOI:
10.1371/journal.pone.0258830.t001
DOI:
10.1371/journal.pone.0258830.t002
DOI:
10.1371/journal.pone.0258830.t003
DOI:
10.1371/journal.pone.0258830.t004
DOI:
10.1371/journal.pone.0258830.t005
DOI:
10.1371/journal.pone.0258830.s001
DOI:
10.1371/journal.pone.0258830.s002
DOI:
10.1371/journal.pone.0258830.s003
DOI:
10.1371/journal.pone.0258830.s004
DOI:
10.1371/journal.pone.0258830.s005
DOI:
10.1371/journal.pone.0258830.r001
DOI:
10.1371/journal.pone.0258830.r002
DOI:
10.1371/journal.pone.0258830.r003
DOI:
10.1371/journal.pone.0258830.r004
DOI:
10.1371/journal.pone.0258830.r005
DOI:
10.1371/journal.pone.0258830.r006
Language:
English
Publisher:
Public Library of Science (PLoS)
Publication Date:
2021
detail.hit.zdb_id:
2267670-3
Permalink