Cargando…

Rapamycin Alleviates Protein Aggregates, Reduces Neuroinflammation, and Rescues Demyelination in Globoid Cell Leukodystrophy

We have shown in vivo and in vitro previously that psychosine causes dysfunction of autophagy and the ubiquitin-proteasome system underlying the pathogenesis of globoid cell leukodystrophy (GLD), a devastating lysosomal storage disease complicated by global demyelination. Here, we investigated the t...

Descripción completa

Detalles Bibliográficos
Autores principales:	Lin, Dar-Shong, Huang, Yu-Wen, Lee, Tsung-Han, Chang, Lung, Huang, Zon-Darr, Wu, Tsu-Yen, Wang, Tuan-Jen, Ho, Che-Sheng
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	MDPI 2023
Materias:	Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10093124/ https://www.ncbi.nlm.nih.gov/pubmed/37048066 http://dx.doi.org/10.3390/cells12070993

Ejemplares similares

Impairment of Proteasome and Autophagy Underlying the Pathogenesis of Leukodystrophy
por: Lin, Dar-Shong, et al.
Publicado: (2020)

Globoid Cell Leukodystrophy (Krabbe Disease): An Update
por: Maghazachi, Azzam A
Publicado: (2023)

Exonic deletions in GALC are frequent in Japanese globoid-cell leukodystrophy patients
por: Irahara-Miyana, Kaori, et al.
Publicado: (2018)

A neglected neurodegenerative disease: Adult-onset globoid cell leukodystrophy
por: Wu, Guode, et al.
Publicado: (2022)

Bone Marrow Transplantation Alters the Tremor Phenotype in the Murine Model of Globoid-Cell Leukodystrophy
por: Reddy, Adarsh S., et al.
Publicado: (2012)

Combined gene/cell therapies provide long-term and pervasive rescue of multiple pathological symptoms in a murine model of globoid cell leukodystrophy
por: Ricca, Alessandra, et al.
Publicado: (2015)

Central Nervous System Pathology Progresses Independently of KC and CXCR2 in Globoid-Cell Leukodystrophy
por: Reddy, Adarsh S., et al.
Publicado: (2013)

Transplantation of mouse embryonic stem cell-derived oligodendrocytes in the murine model of globoid cell leukodystrophy
por: Kuai, Xiao Ling, et al.
Publicado: (2015)

CD8(+) T cell depletion prevents neuropathology in a mouse model of globoid cell leukodystrophy
por: Sutter, Pearl A., et al.
Publicado: (2023)

Novel genetic variant associated with globoid cell leukodystrophy in a family of mixed breed dogs
por: Hammack, Samantha, et al.
Publicado: (2023)

Impact of Mitochondrial A3243G Heteroplasmy on Mitochondrial Bioenergetics and Dynamics of Directly Reprogrammed MELAS Neurons
por: Lin, Dar-Shong, et al.
Publicado: (2022)

Olaparib Attenuates Demyelination and Neuroinflammation in an Organotypic Slice Culture Model of Metachromatic Leukodystrophy
por: Mekhaeil, Marianna, et al.
Publicado: (2023)

Design of a regulated lentiviral vector for hematopoietic stem cell gene therapy of globoid cell leukodystrophy
por: Ungari, Silvia, et al.
Publicado: (2015)

Neural Stem Cell Gene Therapy Ameliorates Pathology and Function in a Mouse Model of Globoid Cell Leukodystrophy
por: Neri, Margherita, et al.
Publicado: (2011)

Therapeutic benefit of lentiviral-mediated neonatal intracerebral gene therapy in a mouse model of globoid cell leukodystrophy
por: Lattanzi, Annalisa, et al.
Publicado: (2014)

Human iPSC-derived astrocytes generated from donors with globoid cell leukodystrophy display phenotypes associated with disease
por: Lieberman, Richard, et al.
Publicado: (2022)

Identifying altered developmental pathways in human globoid cell leukodystrophy iPSCs-derived NSCs using transcriptome profiling
por: Lv, Yafeng, et al.
Publicado: (2023)

Human iPSC-based neurodevelopmental models of globoid cell leukodystrophy uncover patient- and cell type-specific disease phenotypes
por: Mangiameli, Elisabeth, et al.
Publicado: (2021)

CULTIVATION EXPERIMENTS ON THE GLOBOID BODIES OF POLIOMYELITIS
por: Smillie, Wilson G.
Publicado: (1918)

Demyelination load as predictor for disease progression in juvenile metachromatic leukodystrophy
por: Strölin, Manuel, et al.
Publicado: (2017)

THE CULTIVATION AND IMMUNOLOGICAL REACTIONS OF THE GLOBOID BODIES IN POLIOMYELITIS
por: Amoss, Harold L.
Publicado: (1917)

Clinicopathological Features of Globoid Cell Leucodystrophy in Cats
por: Salvadori, C., et al.
Publicado: (2005)

Identification of a Molecular Target of Psychosine and Its Role in Globoid Cell Formation
por: Im, Dong-Soon, et al.
Publicado: (2001)

GLOBOID STRUCTURES IN THE CYTOPLASM OF RAPIDLY GROWING HELA CELLS
por: Harford, Carl G., et al.
Publicado: (1956)

Globoids and Phytase: The Mineral Storage and Release System in Seeds
por: Madsen, Claus Krogh, et al.
Publicado: (2020)

Oxidative Insults and Mitochondrial DNA Mutation Promote Enhanced Autophagy and Mitophagy Compromising Cell Viability in Pluripotent Cell Model of Mitochondrial Disease
por: Lin, Dar-Shong, et al.
Publicado: (2019)

HIV fragments detected in Kaposi sarcoma tumor cells in HIV-infected patients
por: Chen, Tung-Ying, et al.
Publicado: (2022)

Aggregation of MBP in chronic demyelination
por: Frid, Kati, et al.
Publicado: (2015)

Rapamycin inhibits lipopolysaccharide-induced neuroinflammation in vitro and in vivo
por: Mengke, Na-Shun, et al.
Publicado: (2016)

Huperzine—A Improved Animal Behavior in Cuprizone-Induced Mouse Model by Alleviating Demyelination and Neuroinflammation
por: Zhang, Hongyu, et al.
Publicado: (2022)

Human Endogenous Retrovirus and Neuroinflammation in Chronic Inflammatory Demyelinating Polyradiculoneuropathy
por: Faucard, Raphaël, et al.
Publicado: (2016)

Is it Time to Study Leukodystrophies?
por: Graziano, Adriana Carol Eleonora
Publicado: (2017)

Quantitative MRI in leukodystrophies
por: Stellingwerff, Menno D., et al.
Publicado: (2023)

Rapamycin Rescues the Poor Developmental Capacity of Aged Porcine Oocytes
por: Lee, Seung Eun, et al.
Publicado: (2014)

Fingolimod Rescues Demyelination in a Mouse Model of Krabbe's Disease
por: Béchet, Sibylle, et al.
Publicado: (2020)

Nalfurafine reduces neuroinflammation and drives remyelination in models of CNS demyelinating disease
por: Denny, Lisa, et al.
Publicado: (2021)

Late-Onset Leukodystrophy Mimicking Hereditary Spastic Paraplegia without Diffuse Leukodystrophy on Neuroimaging
por: Zhang, Tongxia, et al.
Publicado: (2021)

New insights into globoids of protein storage vacuoles in wheat aleurone using synchrotron soft X-ray microscopy
por: Regvar, Marjana, et al.
Publicado: (2011)

Preventable infections in children with leukodystrophy
por: Anderson, Holly M, et al.
Publicado: (2014)

Intrathecal baclofen in metachromatic leukodystrophy
por: van der Veldt, Nikki, et al.
Publicado: (2018)

Cannot write session to /tmp/vufind_sessions/sess_05v50sti5tr65db7mokrf4d242