Cargando…

Blood–Brain Barrier Integrity Is Perturbed in a Mecp2-Null Mouse Model of Rett Syndrome

Rett syndrome (RTT, online MIM 312750) is a devastating neurodevelopmental disorder characterized by motor and cognitive disabilities. It is mainly caused by pathogenetic variants in the X-linked MECP2 gene, encoding an epigenetic factor crucial for brain functioning. Despite intensive studies, the...

Descripción completa

Detalles Bibliográficos
Autores principales:	Pepe, Giuseppe, Fioriniello, Salvatore, Marracino, Federico, Capocci, Luca, Maglione, Vittorio, D’Esposito, Maurizio, Di Pardo, Alba, Della Ragione, Floriana
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	MDPI 2023
Materias:	Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10136210/ https://www.ncbi.nlm.nih.gov/pubmed/37189354 http://dx.doi.org/10.3390/biom13040606

Ejemplares similares

Transcriptomic and Epigenomic Landscape in Rett Syndrome
por: Marano, Domenico, et al.
Publicado: (2021)

MeCP2 and Major Satellite Forward RNA Cooperate for Pericentric Heterochromatin Organization
por: Fioriniello, Salvatore, et al.
Publicado: (2020)

ATRX Contributes to MeCP2-Mediated Pericentric Heterochromatin Organization during Neural Differentiation
por: Marano, Domenico, et al.
Publicado: (2019)

Oxidative brain damage in Mecp2-mutant murine models of Rett syndrome
por: De Felice, Claudio, et al.
Publicado: (2014)

MeCP2 as a genome-wide modulator: the renewal of an old story
por: Ragione, Floriana Della, et al.
Publicado: (2012)

Epigenetic Factors that Control Pericentric Heterochromatin Organization in Mammals
por: Fioriniello, Salvatore, et al.
Publicado: (2020)

Brain Region and Cell Compartment Dependent Regulation of Electron Transport System Components in Huntington’s Disease Model Mice
por: Burtscher, Johannes, et al.
Publicado: (2021)

Treatment with the Glycosphingolipid Modulator THI Rescues Myelin Integrity in the Striatum of R6/2 HD Mice
por: Pepe, Giuseppe, et al.
Publicado: (2023)

Mecp2-Null Mice Provide New Neuronal Targets for Rett Syndrome
por: Urdinguio, Rocio G., et al.
Publicado: (2008)

MeCP2 Dependent Heterochromatin Reorganization during Neural Differentiation of a Novel Mecp2-Deficient Embryonic Stem Cell Reporter Line
por: Bertulat, Bianca, et al.
Publicado: (2012)

Severe changes in colon epithelium in the Mecp2-null mouse model of Rett syndrome
por: Millar-Büchner, Pamela, et al.
Publicado: (2016)

Pathogenesis of Lethal Aspiration Pneumonia in Mecp2-null Mouse Model for Rett Syndrome
por: Kida, Hiroshi, et al.
Publicado: (2017)

Impairment of blood-brain barrier is an early event in R6/2 mouse model of Huntington Disease
por: Di Pardo, Alba, et al.
Publicado: (2017)

MECP2 Duplication Syndrome: Evidence of Enhanced Oxidative Stress. A Comparison with Rett Syndrome
por: Signorini, Cinzia, et al.
Publicado: (2016)

Differential Expression of Sphingolipid Metabolizing Enzymes in Spontaneously Hypertensive Rats: A Possible Substrate for Susceptibility to Brain and Kidney Damage
por: Pepe, Giuseppe, et al.
Publicado: (2021)

Suppressor mutations in Mecp2-null mice implicate the DNA damage response in Rett syndrome pathology
por: Enikanolaiye, Adebola, et al.
Publicado: (2020)

Stimulation of Sphingosine Kinase 1 (SPHK1) Is Beneficial in a Huntington’s Disease Pre-clinical Model
por: Di Pardo, Alba, et al.
Publicado: (2019)

Reduction of aberrant NF-κB signalling ameliorates Rett syndrome phenotypes in Mecp2-null mice
por: Kishi, Noriyuki, et al.
Publicado: (2016)

QT(c) interval and ventricular action potential prolongation in the Mecp2 ( Null/+) murine model of Rett syndrome
por: Cheng, Hongwei, et al.
Publicado: (2022)

Non-coding RNAs in chromatin disease involving neurological defects
por: Della Ragione, Floriana, et al.
Publicado: (2014)

Alterations of Gene Expression and Glutamate Clearance in Astrocytes Derived from an MeCP2-Null Mouse Model of Rett Syndrome
por: Okabe, Yasunori, et al.
Publicado: (2012)

Isolation of MECP2-null Rett Syndrome patient hiPS cells and isogenic controls through X-chromosome inactivation
por: Cheung, Aaron Y.L., et al.
Publicado: (2011)

Rett Syndrome and MECP2 Duplication Syndrome: Disorders of MeCP2 Dosage
por: Collins, Bridget E, et al.
Publicado: (2022)

The relationship of Rett syndrome and MECP2 disorders to autism
por: Neul, Jeffrey Lorenz
Publicado: (2012)

Multimodal Neuroimaging in Rett Syndrome With MECP2 Mutation
por: Kong, Yu, et al.
Publicado: (2022)

Comprehensive Volumetric Analysis of Mecp2-Null Mouse Model for Rett Syndrome by T2-Weighted 3D Magnetic Resonance Imaging
por: Akaba, Yuichi, et al.
Publicado: (2022)

Reduced brain UCP2 expression mediated by microRNA-503 contributes to increased stroke susceptibility in the high-salt fed stroke-prone spontaneously hypertensive rat
por: Rubattu, Speranza, et al.
Publicado: (2017)

De novo Synthesis of Sphingolipids Is Defective in Experimental Models of Huntington's Disease
por: Di Pardo, Alba, et al.
Publicado: (2017)

Spectrum of MECP2 mutations in Vietnamese patients with RETT syndrome
por: Le Thi Thanh, Huong, et al.
Publicado: (2018)

Structural investigation of Rett-inducing MeCP2 mutations()
por: Spiga, Ottavia, et al.
Publicado: (2018)

Rett mutations attenuate phase separation of MeCP2
por: Fan, Chunyan, et al.
Publicado: (2020)

The Molecular Functions of MeCP2 in Rett Syndrome Pathology
por: Sharifi, Osman, et al.
Publicado: (2021)

MeCP2: The Genetic Driver of Rett Syndrome Epigenetics
por: Good, Katrina V., et al.
Publicado: (2021)

Effects of Mecp2 loss of function in embryonic cortical neurons: a bioinformatics strategy to sort out non-neuronal cells variability from transcriptome profiling
por: Vacca, Marcella, et al.
Publicado: (2016)

Effects of early‐life exposure to THIP on brainstem neuronal excitability in the Mecp2‐null mouse model of Rett syndrome before and after drug withdrawal
por: Zhong, Weiwei, et al.
Publicado: (2017)

When Rett syndrome is due to genes other than MECP2
por: Percy, Alan K., et al.
Publicado: (2018)

Genetic Analysis of MECP2 Gene in Iranian Patients with Rett Syndrome
por: NASIRI, Jafar, et al.
Publicado: (2019)

Characterization of Rett Syndrome-like phenotypes in Mecp2-knockout rats
por: Wu, Yang, et al.
Publicado: (2016)

Rett Syndrome in Males: The Different Clinical Course in Two Brothers with the Same Microduplication MECP2 Xq28
por: Pitzianti, Maria Bernarda, et al.
Publicado: (2019)

Rett syndrome – biological pathways leading from MECP2 to disorder phenotypes
por: Ehrhart, Friederike, et al.
Publicado: (2016)

Cannot write session to /tmp/vufind_sessions/sess_rvibols67o826oio31cjjki7qa