Cargando…

Differential response of the liver to bile acid treatment in a mouse model of Niemann-Pick disease type C

Niemann-Pick disease type C (NPC) disease is a neurodegenerative lysosomal storage disease caused by mutations in the NPC1 or NPC2 genes. Liver disease is also a common feature of NPC that can present as cholestatic jaundice in the neonatal period. Liver enzymes can remain elevated above the normal...

Descripción completa

Detalles Bibliográficos
Autores principales:	Nicoli, Elena-Raluca, Huebecker, Mylene, Smith, David, Morris, Lauren, Platt, Frances M.
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	F1000 Research Limited 2018
Materias:	Research Note
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5639931/ https://www.ncbi.nlm.nih.gov/pubmed/29062912 http://dx.doi.org/10.12688/wellcomeopenres.12431.2

Ejemplares similares

Identification of novel bile acids as biomarkers for the early diagnosis of Niemann‐Pick C disease
por: Mazzacuva, Francesca, et al.
Publicado: (2016)

Case Report: Ursodeoxycholic acid treatment in Niemann-Pick disease type C; clinical experience in four cases
por: Evans, William R.H., et al.
Publicado: (2017)

AAV9 intracerebroventricular gene therapy improves lifespan, locomotor function and pathology in a mouse model of Niemann–Pick type C1 disease
por: Hughes, Michael P, et al.
Publicado: (2018)

Defective iron homeostasis and hematological abnormalities in Niemann-Pick disease type C1
por: Chen, Oscar C W, et al.
Publicado: (2023)

Genetic background modifies phenotypic severity and longevity in a mouse model of Niemann-Pick disease type C1
por: Rodriguez-Gil, Jorge L., et al.
Publicado: (2020)

Immune dysfunction in Niemann‐Pick disease type C
por: Platt, Nick, et al.
Publicado: (2015)

Defective Cytochrome P450-Catalysed Drug Metabolism in Niemann-Pick Type C Disease
por: Nicoli, Elena-Raluca, et al.
Publicado: (2016)

Circadian profiling in two mouse models of lysosomal storage disorders; Niemann Pick type-C and Sandhoff disease
por: Richardson, Katie, et al.
Publicado: (2016)

NMR analysis reveals significant differences in the plasma metabolic profiles of Niemann Pick C1 patients, heterozygous carriers, and healthy controls
por: Probert, Fay, et al.
Publicado: (2017)

Defective platelet function in Niemann‐Pick disease type C1
por: Chen, Oscar C. W., et al.
Publicado: (2020)

The expanding boundaries of sphingolipid lysosomal storage diseases; insights from Niemann–Pick disease type C
por: Platt, Frances M.
Publicado: (2023)

Abnormal LAMP1 glycosylation may play a role in Niemann-Pick disease, type C pathology
por: Cawley, Niamh X., et al.
Publicado: (2020)

Mechanistic convergence and shared therapeutic targets in Niemann‐Pick disease
por: Colaco, Alexandria, et al.
Publicado: (2019)

Imaging of changes in copper trafficking and redistribution in a mouse model of Niemann-Pick C disease using positron emission tomography
por: Baguña Torres, Julia, et al.
Publicado: (2019)

Inhibition of the Niemann-Pick C1 protein is a conserved feature of multiple strains of pathogenic mycobacteria
por: Weng, Yuzhe, et al.
Publicado: (2022)

Niemann-Pick disease type C
por: Vanier, Marie T
Publicado: (2010)

Stem Cells and Niemann Pick Disease
por: Andolina, Marino
Publicado: (2014)

Stem Cells in Niemann-Pick Disease
por: Kim, Sun-Jung, et al.
Publicado: (2008)

Niemann-Pick disease and platelet dysfunction
Publicado: (2012)

Elevation of plasma lysosphingomyelin-509 and urinary bile acid metabolite in Niemann-Pick disease type C-affected individuals
por: Mashima, Ryuichi, et al.
Publicado: (2018)

Drug‐induced increase in lysobisphosphatidic acid reduces the cholesterol overload in Niemann–Pick type C cells and mice
por: Moreau, Dimitri, et al.
Publicado: (2019)

Unbiased yeast screens identify cellular pathways affected in Niemann–Pick disease type C
por: Colaco, Alexandria, et al.
Publicado: (2020)

Early glial activation, synaptic changes and axonal pathology in the thalamocortical system of Niemann–Pick type C1 mice
por: Pressey, Sarah N.R., et al.
Publicado: (2012)

A Novel Small NPC1 Promoter Enhances AAV-Mediated Gene Therapy in Mouse Models of Niemann–Pick Type C1 Disease
por: Hughes, Michael Paul, et al.
Publicado: (2023)

Gadolinium Chloride Rescues Niemann–Pick Type C Liver Damage
por: Klein, Andrés D., et al.
Publicado: (2018)

Anti-TNF therapy for inflammatory bowel disease in patients with neurodegenerative Niemann-Pick disease Type C
por: Williams, Isabelle, et al.
Publicado: (2022)

Complement Component C3 Participates in Early Stages of Niemann–Pick C Mouse Liver Damage
por: Klein, Andrés D., et al.
Publicado: (2020)

Lysosomal and Mitochondrial Liaisons in Niemann-Pick Disease
por: Torres, Sandra, et al.
Publicado: (2017)

Niemann-Pick Disease: An Approach for Diagnosis in Adulthood
por: Patiño-Escobar, Bonell, et al.
Publicado: (2019)

A novel, highly sensitive and specific biomarker for Niemann-Pick type C1 disease
por: Giese, Anne-Katrin, et al.
Publicado: (2015)

Molecular basis for a new bovine model of Niemann-Pick type C disease
por: Woolley, Shernae A., et al.
Publicado: (2020)

GCase and LIMP2 Abnormalities in the Liver of Niemann Pick Type C Mice
por: van der Lienden, Martijn J. C., et al.
Publicado: (2021)

The prognostic value of Niemann-Pick C1-like protein 1 and Niemann-Pick disease type C2 in hepatocellular carcinoma
por: Chen, Ke-Ji, et al.
Publicado: (2018)

Clinical disease characteristics of patients with Niemann-Pick Disease Type C: findings from the International Niemann-Pick Disease Registry (INPDR)
por: Bolton, Shaun C., et al.
Publicado: (2022)

Evaluation of an Anti-Tumor Necrosis Factor Therapeutic in a Mouse Model of Niemann-Pick C Liver Disease
por: Vincent, Melanie, et al.
Publicado: (2010)

Evaluation of Two Liver Treatment Strategies in a Mouse Model of Niemann–Pick-Disease Type C1
por: Ebner, Lynn, et al.
Publicado: (2018)

Elevated Alpha-Fetoprotein in Infantile-Onset Niemann-Pick Type C Disease with Liver Involvement
por: Kraus, Dror, et al.
Publicado: (2022)

Clear Cell Chondrosarcoma in Association With Niemann-Pick Disease
por: Srikanth, K. N., et al.
Publicado: (2005)

Anesthetic Management in a Child With Niemann-Pick Disease
por: Espahbodi, Ebrahim, et al.
Publicado: (2016)

Niemann-Pick Disease: An Underdiagnosed Lysosomal Storage Disorder
por: Panigrahi, Inusha, et al.
Publicado: (2019)

Cannot write session to /tmp/vufind_sessions/sess_a110mukrb8vtfb1bfgk9gvgoj5