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

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...

Πλήρης περιγραφή

Λεπτομέρειες βιβλιογραφικής εγγραφής
Κύριοι συγγραφείς:	Nicoli, E, Smith, D, Morris, L, Platt, F
Μορφή:	Journal article
Γλώσσα:	English
Έκδοση:	F1000Research 2017

Παρόμοια τεκμήρια

Differential response of the liver to bile acid treatment in a mouse model of Niemann-Pick disease type C [version 2; referees: 2 approved, 1 not approved]
ανά: Elena-Raluca Nicoli, κ.ά.
Έκδοση: (2018-04-01)

Case Report: Ursodeoxycholic acid treatment in Niemann-Pick disease type C; clinical experience in four cases
ανά: Evans, W, κ.ά.
Έκδοση: (2017)

Niemann-Pick Disease Type C
ανά: J Gordon Millichap
Έκδοση: (1989-09-01)

Niemann-Pick disease type C
ανά: Vanier Marie T
Έκδοση: (2010-06-01)

Beneficial effects of anti-inflammatory therapy in a mouse model of Niemann-Pick disease type C1.
ανά: Smith, D, κ.ά.
Έκδοση: (2009)

Circadian profiling in two mouse models of lysosomal storage disorders; Niemann Pick type-C and Sandhoff disease.
ανά: Richardson, K, κ.ά.
Έκδοση: (2016)

Defective platelet function in Niemann‐Pick disease type C1
ανά: Chen, OCW, κ.ά.
Έκδοση: (2020)

Defective Cytochrome P450-Catalysed Drug Metabolism in Niemann-Pick Type C Disease
ανά: Platt, F, κ.ά.
Έκδοση: (2016)

Defective Cytochrome P450-Catalysed Drug Metabolism in Niemann-Pick Type C Disease
ανά: Nicoli, E, κ.ά.
Έκδοση: (2016)

Defective Cytochrome P450-Catalysed Drug Metabolism in Niemann-Pick Type C Disease
ανά: Platt, F, κ.ά.
Έκδοση: (2016)

Lipids on trial: the search for the offending metabolite in Niemann-Pick type C disease.
ανά: Lloyd-Evans, E, κ.ά.
Έκδοση: (2010)

A new surrogate marker for CNS pathology in Niemann-Pick disease type C?
ανά: Platt, F, κ.ά.
Έκδοση: (2009)

Niemann-Pick type c disease: pathogenesis and therapy
ανά: Colaco, A
Έκδοση: (2016)

A case of Niemann – Pick disease type C
ανά: Sergei Anatolyevich Klyushnikov, κ.ά.
Έκδοση: (2013-12-01)

Difficult diagnosis. Niemann - Pick disease, type C
ανά: L. S. Namazova-baranova, κ.ά.
Έκδοση: (2014-12-01)

Niemann-Pick disease type C with kidney involvement
ανά: Aanand A. Patel, κ.ά.
Έκδοση: (2021-03-01)

Genetic background modifies phenotypic severity and longevity in a mouse model of Niemann-Pick disease type C1
ανά: Rodriguez-Gil, J, κ.ά.
Έκδοση: (2020)

Case Report: Ursodeoxycholic acid treatment in Niemann-Pick disease type C; clinical experience in four cases [version 1; referees: 2 approved]
ανά: William R.H. Evans, κ.ά.
Έκδοση: (2017-08-01)

Plasma phosphorylated-tau217 is increased in Niemann–Pick disease type C
ανά: Gonzalez-Ortiz, F, κ.ά.
Έκδοση: (2024)

Anti-TNF therapy for inflammatory bowel disease in patients with neurodegenerative Niemann-Pick disease Type C
ανά: Williams, I, κ.ά.
Έκδοση: (2022)

Unbiased yeast screens identify cellular pathways affected in Niemann–Pick disease type C
ανά: Colaco, A, κ.ά.
Έκδοση: (2020)

Evaluation of new therapies in Niemann-Pick type C disease
ανά: Al Eisa, N
Έκδοση: (2014)

Discovering genetic modifiers in Niemann-Pick Disease Type C
ανά: Rodriguez-Gil, J
Έκδοση: (2019)

Hypometabolism and atrophy patterns associated with Niemann-Pick type C
ανά: Jesús Silva-Rodríguez, κ.ά.
Έκδοση: (2025-02-01)

RECOMMENDATIONS FOR DIAGNOSTICS AND TREATMENT OF NIEMANN-PICK TYPE C DISEASE
ανά: James E. Wraith, κ.ά.
Έκδοση: (2010-02-01)

RECOMMENDATIONS FOR DIAGNOSTICS AND TREATMENT OF NIEMANN-PICK TYPE C DISEASE
ανά: James E. Wraith, κ.ά.
Έκδοση: (2010-02-01)

Estimated prevalence of Niemann–Pick type C disease in Quebec
ανά: Marjorie Labrecque, κ.ά.
Έκδοση: (2021-11-01)

Niemann-Pick disease
ανά: Serap Karaman, κ.ά.
Έκδοση: (2011-03-01)

Niemann-Pick disease
ανά: Serap Karaman, κ.ά.
Έκδοση: (2011-03-01)

Treatment with miglustat reverses the lipid-trafficking defect in Niemann-Pick disease type C.
ανά: Lachmann, R, κ.ά.
Έκδοση: (2004)

Drug‐induced increase in lysobisphosphatidic acid reduces the cholesterol overload in Niemann–Pick type C cells and mice
ανά: Moreau, D, κ.ά.
Έκδοση: (2019)

Elevated Alpha-Fetoprotein in Infantile-Onset Niemann-Pick Type C Disease with Liver Involvement
ανά: Dror Kraus, κ.ά.
Έκδοση: (2022-04-01)

AAV9 intracerebroventricular gene therapy improves lifespan, locomotor function and pathology in a mouse model of Niemann-Pick type C1 disease
ανά: Hughes, MP, κ.ά.
Έκδοση: (2018)

Early glial activation, synaptic changes and axonal pathology in the thalamocortical system of Niemann-Pick type C1 mice
ανά: Pressey, S, κ.ά.
Έκδοση: (2012)

Early glial activation, synaptic changes and axonal pathology in the thalamocortical system of Niemann-Pick type C1 mice.
ανά: Pressey, SN, κ.ά.
Έκδοση: (2012)

Treatment with miglustat reverses the lipid-trafficking defect in Niemann–Pick disease type C
ανά: Robin H Lachmann, κ.ά.
Έκδοση: (2004-08-01)

Genetic background modifies phenotypic severity and longevity in a mouse model of Niemann-Pick disease type C1
ανά: Jorge L. Rodriguez-Gil, κ.ά.
Έκδοση: (2020-03-01)

Accumulation of glycosphingolipids in Niemann-Pick C disease disrupts endosomal transport.
ανά: te Vruchte, D, κ.ά.
Έκδοση: (2004)

Molecular basis for a new bovine model of Niemann-Pick type C disease
ανά: Woolley, SA, κ.ά.
Έκδοση: (2020)

RECOMMENDATIONS ON DIAGNOSTICS AND TACTICS OF MANAGEMENT OF NIEMANN-PICK DISEASE, TYPE C
ανά: L. M. Kuzenkova, κ.ά.
Έκδοση: (2012-12-01)