Cargando…

Inhibition of β-Glucocerebrosidase Activity Preserves Motor Unit Integrity in a Mouse Model of Amyotrophic Lateral Sclerosis

Recent metabolomic reports connect dysregulation of glycosphingolipids, particularly ceramide and glucosylceramide, to neurodegeneration and to motor unit dismantling in amyotrophic lateral sclerosis at late disease stage. We report here altered levels of gangliosides in the cerebrospinal fluid of a...

Descripción completa

Detalles Bibliográficos
Autores principales:	Henriques, Alexandre, Huebecker, Mylene, Blasco, Hélène, Keime, Céline, Andres, Christian R., Corcia, Philippe, Priestman, David A., Platt, Frances M., Spedding, Michael, Loeffler, Jean-Philippe
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Nature Publishing Group UK 2017
Materias:	Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5507914/ https://www.ncbi.nlm.nih.gov/pubmed/28701774 http://dx.doi.org/10.1038/s41598-017-05313-0

Ejemplares similares

Amyotrophic lateral sclerosis and denervation alter sphingolipids and up-regulate glucosylceramide synthase
por: Henriques, Alexandre, et al.
Publicado: (2015)

Sphingolipid Metabolism Is Dysregulated at Transcriptomic and Metabolic Levels in the Spinal Cord of an Animal Model of Amyotrophic Lateral Sclerosis
por: Henriques, Alexandre, et al.
Publicado: (2018)

Glycosphingolipid levels and glucocerebrosidase activity are altered in normal aging of the mouse brain
por: Hallett, Penelope J., et al.
Publicado: (2018)

Ambroxol Hydrochloride Improves Motor Functions and Extends Survival in a Mouse Model of Familial Amyotrophic Lateral Sclerosis
por: Bouscary, Alexandra, et al.
Publicado: (2019)

Blood Cell Palmitoleate-Palmitate Ratio Is an Independent Prognostic Factor for Amyotrophic Lateral Sclerosis
por: Henriques, Alexandre, et al.
Publicado: (2015)

Is There a Role for Vitamin D in Amyotrophic Lateral Sclerosis? A Systematic Review and Meta-Analysis
por: Lanznaster, Débora, et al.
Publicado: (2020)

Metabolomics: A Tool to Understand the Impact of Genetic Mutations in Amyotrophic Lateral Sclerosis
por: Lanznaster, Débora, et al.
Publicado: (2020)

Amyotrophic lateral sclerosis and the clinical potential of dexpramipexole
por: Corcia, Philippe, et al.
Publicado: (2012)

Current view and perspectives in amyotrophic lateral sclerosis
por: Mathis, Stéphane, et al.
Publicado: (2017)

The debated toxic role of aggregated TDP-43 in amyotrophic lateral sclerosis: a resolution in sight?
por: Hergesheimer, Rudolf C, et al.
Publicado: (2019)

Aβ1-42 and Tau as Potential Biomarkers for Diagnosis and Prognosis of Amyotrophic Lateral Sclerosis
por: Lanznaster, Débora, et al.
Publicado: (2020)

Implication of Central Nervous System Barrier Impairment in Amyotrophic Lateral Sclerosis: Gender-Related Difference in Patients
por: Alarcan, Hugo, et al.
Publicado: (2023)

Some CSF Kynurenine Pathway Intermediates Associated with Disease Evolution in Amyotrophic Lateral Sclerosis
por: Alarcan, Hugo, et al.
Publicado: (2021)

Dysregulations of Expression of Genes of the Ubiquitin/SUMO Pathways in an In Vitro Model of Amyotrophic Lateral Sclerosis Combining Oxidative Stress and SOD1 Gene Mutation
por: Dangoumau, Audrey, et al.
Publicado: (2021)

The Role of Skeletal Muscle in Amyotrophic Lateral Sclerosis
por: Loeffler, Jean‐Philippe, et al.
Publicado: (2016)

(1)H-NMR-Based Metabolomic Profiling of CSF in Early Amyotrophic Lateral Sclerosis
por: Blasco, Hélène, et al.
Publicado: (2010)

Study of Ubiquitin Pathway Genes in a French Population with Amyotrophic Lateral Sclerosis: Focus on HECW1 Encoding the E3 Ligase NEDL1
por: Haouari, Shanez, et al.
Publicado: (2023)

Identification of genetic modifiers of murine hepatic β-glucocerebrosidase activity
por: Durán, Anyelo, et al.
Publicado: (2021)

Elevated Serum Ferritin Is Associated with Reduced Survival in Amyotrophic Lateral Sclerosis
por: Nadjar, Yann, et al.
Publicado: (2012)

Correction: (1)H-NMR-Based Metabolomic Profiling of CSF in Early Amyotrophic Lateral Sclerosis
por: Blasco, Hélène, et al.
Publicado: (2010)

Metabolic Profile and Pathological Alterations in the Muscle of Patients with Early-Stage Amyotrophic Lateral Sclerosis
por: Lanznaster, Débora, et al.
Publicado: (2022)

Molecular Imaging of Microglial Activation in Amyotrophic Lateral Sclerosis
por: Corcia, Philippe, et al.
Publicado: (2012)

A ferroptosis–based panel of prognostic biomarkers for Amyotrophic Lateral Sclerosis
por: Devos, David, et al.
Publicado: (2019)

Sex-dependent effects of chromogranin B P413L allelic variant as disease modifier in amyotrophic lateral sclerosis
por: Ohta, Yasuyuki, et al.
Publicado: (2016)

Platelet Serotonin Level Predicts Survival in Amyotrophic Lateral Sclerosis
por: Dupuis, Luc, et al.
Publicado: (2010)

TDP-43-Mediated Toxicity in HEK293T Cells: A Fast and Reproducible Protocol To Be Employed in the Search of New Therapeutic Options against Amyotrophic Lateral Sclerosis
por: Lanznaster, Débora, et al.
Publicado: (2019)

Reduced sphingolipid hydrolase activities, substrate accumulation and ganglioside decline in Parkinson’s disease
por: Huebecker, Mylene, et al.
Publicado: (2019)

Correction to: Reduced sphingolipid hydrolase activities, substrate accumulation and ganglioside decline in Parkinson’s disease
por: Huebecker, Mylene, et al.
Publicado: (2020)

Author Correction: A ferroptosis–based panel of prognostic biomarkers for Amyotrophic Lateral Sclerosis
por: Devos, David, et al.
Publicado: (2020)

Prionoids in amyotrophic lateral sclerosis
por: Gosset, Philippe, et al.
Publicado: (2022)

CXCL13/CXCR5 signalling is pivotal to preserve motor neurons in amyotrophic lateral sclerosis
por: Trolese, Maria Chiara, et al.
Publicado: (2020)

Molecular Motor Proteins and Amyotrophic Lateral Sclerosis
por: Soo, Kai Y, et al.
Publicado: (2011)

Glycosphingolipid metabolism and its role in ageing and Parkinson’s disease
por: Wallom, Kerri-Lee, et al.
Publicado: (2021)

Could Conservative Iron Chelation Lead to Neuroprotection in Amyotrophic Lateral Sclerosis?
por: Moreau, Caroline, et al.
Publicado: (2018)

Tauroursodeoxycholic acid in patients with amyotrophic lateral sclerosis: The TUDCA-ALS trial protocol
por: Albanese, Alberto, et al.
Publicado: (2022)

Altered Expression of Ganglioside Metabolizing Enzymes Results in GM3 Ganglioside Accumulation in Cerebellar Cells of a Mouse Model of Juvenile Neuronal Ceroid Lipofuscinosis
por: Somogyi, Aleksandra, et al.
Publicado: (2018)

Skeletal Muscle Metabolism: Origin or Prognostic Factor for Amyotrophic Lateral Sclerosis (ALS) Development?
por: Quessada, Cyril, et al.
Publicado: (2021)

Impaired Structural Motor Connectome in Amyotrophic Lateral Sclerosis
por: Verstraete, Esther, et al.
Publicado: (2011)

Amyotrophic lateral sclerosis: a dying motor unit?
por: Piotrkiewicz, Maria, et al.
Publicado: (2013)

Regional motor cortex dysfunction in amyotrophic lateral sclerosis
por: Menon, Parvathi, et al.
Publicado: (2019)

Cannot write session to /tmp/vufind_sessions/sess_e8t3bf12ha4qhonn33goc4h9cs