Cargando…

Demyelination and axonal preservation in a transgenic mouse model of Pelizaeus-Merzbacher disease

It is widely thought that demyelination contributes to the degeneration of axons and, in combination with acute inflammatory injury, is responsible for progressive axonal loss and persistent clinical disability in inflammatory demyelinating disease. In this study we sought to characterize the relati...

Descripción completa

Detalles Bibliográficos
Autores principales:	Edgar, Julia M, McCulloch, Mailis C, Montague, Paul, Brown, Angus M, Thilemann, Sebastian, Pratola, Laura, Gruenenfelder, Fredrik I, Griffiths, Ian R, Nave, Klaus-Armin
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	WILEY-VCH Verlag 2010
Materias:	Report
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3377270/ https://www.ncbi.nlm.nih.gov/pubmed/20091761 http://dx.doi.org/10.1002/emmm.200900057

Ejemplares similares

Neural stem cells restore myelin in a demyelinating model of Pelizaeus-Merzbacher disease
por: Gruenenfelder, Fredrik I, et al.
Publicado: (2020)

Ketogenic diet ameliorates axonal defects and promotes myelination in Pelizaeus–Merzbacher disease
por: Stumpf, Sina K., et al.
Publicado: (2019)

Curcumin therapy in a Plp1 transgenic mouse model of Pelizaeus-Merzbacher disease
por: Epplen, Dirk B, et al.
Publicado: (2015)

Pelizaeus-Merzbacher disease in siblings
por: Mittal, Amit, et al.
Publicado: (2010)

Correction to: Ketogenic diet ameliorates axonal defects and promotes myelination in Pelizaeus–Merzbacher disease
por: Stumpf, Sina K., et al.
Publicado: (2019)

Suppression of proteolipid protein rescues Pelizaeus-Merzbacher disease
por: Elitt, Matthew S., et al.
Publicado: (2020)

Clinical and mutational spectrum of Colombian patients with Pelizaeus Merzbacher Disease
por: Velasco Parra, Harvy Mauricio, et al.
Publicado: (2018)

Optical genome mapping in an atypical Pelizaeus-Merzbacher prenatal challenge
por: Rogac, Mihael, et al.
Publicado: (2023)

Overexpression of the myelin proteolipid protein leads to accumulation of cholesterol and proteolipid protein in endosomes/lysosomes: implications for Pelizaeus-Merzbacher disease
por: Simons, Mikael, et al.
Publicado: (2002)

A genetically proven case of Pelizaeus-Merzbacher disease: Clinicoradiological clues
por: Saini, Lokesh, et al.
Publicado: (2016)

Oxidative stress and mitochondrial dynamics malfunction are linked in Pelizaeus‐Merzbacher disease
por: Ruiz, Montserrat, et al.
Publicado: (2017)

Pelizaeus–Merzbacher Disease: the First Genetically Approved Case Report from Iran
por: Ashrafi, Mahmoud-Reza, et al.
Publicado: (2011)

Potential for Cell-Mediated Immune Responses in Mouse Models of Pelizaeus-Merzbacher Disease
por: Southwood, Cherie M., et al.
Publicado: (2013)

New Mutation of Pelizaeus-Merzbacher-Like Disease; A Report from Iran
por: Karimzadeh, Parvaneh, et al.
Publicado: (2014)

Cellular Pathology of Pelizaeus-Merzbacher Disease Involving Chaperones Associated with Endoplasmic Reticulum Stress
por: Inoue, Ken
Publicado: (2017)

Magnetic Resonance Imaging and Spectroscopy Analysis in a Pelizaeus–Merzbacher Disease Rat Model
por: Ishikawa, Maho, et al.
Publicado: (2022)

Missense mutation of MAL causes a rare leukodystrophy similar to Pelizaeus-Merzbacher disease
por: Elpidorou, Marilena, et al.
Publicado: (2022)

Disrupted Proteolipid Protein Trafficking Results in Oligodendrocyte Apoptosis in an Animal Model of Pelizaeus-Merzbacher Disease
por: Gow, Alexander, et al.
Publicado: (1998)

Unusual Presentation of Pelizaeus-Merzbacher Disease: Female Patient with Deletion of the Proteolipid Protein 1 Gene
por: Brender, Teva, et al.
Publicado: (2015)

Drug screening for Pelizaeus-Merzbacher disease by quantifying the total levels and membrane localization of PLP1
por: Kouga, Takeshi, et al.
Publicado: (2019)

Novel Mutations in NPC1 are Associated with Pelizaeus-Merzbacher-Like Disease: A Case Report
por: Fu, Hongling, et al.
Publicado: (2021)

Genotype–phenotype correlation and natural history analyses in a Chinese cohort with pelizaeus–merzbacher disease
por: Duan, Ruoyu, et al.
Publicado: (2022)

Familial Case of Pelizaeus-Merzbacher Disorder Detected by Oligoarray Comparative Genomic Hybridization: Genotype-to-Phenotype Diagnosis
por: Najafi, Kimia, et al.
Publicado: (2017)

Anesthetic challenges and successful management of a child with Pelizaeus–Merzbacher disease using general and caudal anesthesia
por: Kapoor, Ravish, et al.
Publicado: (2018)

Pelizaeus-Merzbacher Disease in a 4-Year-Old Female Child: “A Rare Case Report”
por: Gagan, Devesh, et al.
Publicado: (2023)

The natural history of Pelizaeus–Merzbacher disease caused by PLP1 duplication: A multiyear case series
por: Trepanier, Angela M., et al.
Publicado: (2023)

Magnetic resonance imaging and spectroscopic analysis in 5 cases of Pelizaeus-Merzbacher disease: metabolic abnormalities as diagnostic tools
por: Lee, Eun, et al.
Publicado: (2012)

Pelizaeus-Merzbacher disease can be a differential diagnosis in males presenting with severe neonatal respiratory distress and hypotonia
por: Ueda, Ayako, et al.
Publicado: (2018)

Deep intronic deletion in intron 3 of PLP1 is associated with a severe phenotype of Pelizaeus-Merzbacher disease
por: Yamamoto-Shimojima, Keiko, et al.
Publicado: (2021)

Prenatal diagnosis of PLP1 duplication by single nucleotide polymorphism array in a family with Pelizaeus-Merzbacher disease
por: Xue, Huili, et al.
Publicado: (2021)

Altered high-energy phosphate and membrane metabolism in Pelizaeus–Merzbacher disease using phosphorus magnetic resonance spectroscopy
por: Laukka, Jeremy J, et al.
Publicado: (2022)

Epidural Anesthesia and Continuous Epidural Analgesia in a Pediatric Patient With Pelizaeus-Merzbacher Disease: A Case Report
por: Maruta, Toyoaki, et al.
Publicado: (2022)

Pathologic and Phenotypic Alterations in a Mouse Expressing a Connexin47 Missense Mutation That Causes Pelizaeus-Merzbacher–Like Disease in Humans
por: Tress, Oliver, et al.
Publicado: (2011)

Targeted metabolomic analyses of cellular models of pelizaeus-merzbacher disease reveal plasmalogen and myo-inositol solute carrier dysfunction
por: Wood, Paul L, et al.
Publicado: (2011)

Chemical Screening Identifies Enhancers of Mutant Oligodendrocyte Survival and Unmasks a Distinct Pathological Phase in Pelizaeus-Merzbacher Disease
por: Elitt, Matthew S., et al.
Publicado: (2018)

Long-Term Safety, Immunologic Response, and Imaging Outcomes following Neural Stem Cell Transplantation for Pelizaeus-Merzbacher Disease
por: Gupta, Nalin, et al.
Publicado: (2019)

Pelizaeus-Merzbacher-Like Disease 1 Caused by a Novel Mutation in GJC2 Gene: A Case Report
por: Javadikooshesh, Sepehr, et al.
Publicado: (2021)

Pelizaeus-Merzbacher Disease with PLP1 Exon 1 Duplication, Previously Misdiagnosed as Cerebral Palsy: a Case Report
por: Lee, Su Ji, et al.
Publicado: (2021)

A Patient With Pelizaeus-Merzbacher Disease Caused by a c.67G>A Mutation in the PLP1 Gene
por: Usman, Mohammad, et al.
Publicado: (2023)

Involvement of ER Stress in Dysmyelination of Pelizaeus-Merzbacher Disease with PLP1 Missense Mutations Shown by iPSC-Derived Oligodendrocytes
por: Numasawa-Kuroiwa, Yuko, et al.
Publicado: (2014)

Cannot write session to /tmp/vufind_sessions/sess_r8hphs70q5hdvroq494l8pmu1b