Cargando…

Gadolinium Chloride Rescues Niemann–Pick Type C Liver Damage

Niemann–Pick type C (NPC) disease is a rare neurovisceral cholesterol storage disorder that arises from loss of function mutations in the NPC1 or NPC2 genes. Soon after birth, some patients present with an aggressive hepatosplenomegaly and cholestatic signs. Histopathologically, the liver presents w...

Descripción completa

Detalles Bibliográficos
Autores principales:	Klein, Andrés D., Oyarzún, Juan Esteban, Cortez, Cristian, Zanlungo, Silvana
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	MDPI 2018
Materias:	Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6274821/ https://www.ncbi.nlm.nih.gov/pubmed/30441844 http://dx.doi.org/10.3390/ijms19113599

Ejemplares similares

Proteomic Analysis of Niemann-Pick Type C Hepatocytes Reveals Potential Therapeutic Targets for Liver Damage
por: Balboa, Elisa, et al.
Publicado: (2021)

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

Oxidative Stress: A Pathogenic Mechanism for Niemann-Pick Type C Disease
por: Vázquez, Mary Carmen, et al.
Publicado: (2012)

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

Understanding the phenotypic variability in Niemann-Pick disease type C (NPC): a need for precision medicine
por: Las Heras, Macarena, et al.
Publicado: (2023)

Genistein Activates Transcription Factor EB and Corrects Niemann–Pick C Phenotype
por: Argüello, Graciela, et al.
Publicado: (2021)

Alteration of Gene Expression Profile in Niemann-Pick Type C Mice Correlates with Tissue Damage and Oxidative Stress
por: Vázquez, Mary C., et al.
Publicado: (2011)

c-Abl Activation Linked to Autophagy-Lysosomal Dysfunction Contributes to Neurological Impairment in Niemann-Pick Type A Disease
por: Marín, Tamara, et al.
Publicado: (2022)

Cholinergic Abnormalities, Endosomal Alterations and Up-Regulation of Nerve Growth Factor Signaling in Niemann-Pick Type C Disease
por: Cabeza, Carolina, et al.
Publicado: (2012)

Disruption in Connexin-Based Communication Is Associated with Intracellular Ca(2+) Signal Alterations in Astrocytes from Niemann-Pick Type C Mice
por: Sáez, Pablo J., et al.
Publicado: (2013)

Neuronal and epithelial cell rescue resolves chronic systemic inflammation in the lipid storage disorder Niemann-Pick C
por: Lopez, Manuel E., et al.
Publicado: (2012)

Complement is dispensable for neurodegeneration in Niemann-Pick disease type C
por: Lopez, Manuel E, et al.
Publicado: (2012)

Vitamin E Dietary Supplementation Improves Neurological Symptoms and Decreases c-Abl/p73 Activation in Niemann-Pick C Mice
por: Marín, Tamara, et al.
Publicado: (2014)

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

Mitochondrial GSH replenishment as a potential therapeutic approach for Niemann Pick type C disease
por: Torres, Sandra, et al.
Publicado: (2016)

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)

Genome sequencing in a case of Niemann–Pick type C
por: Dougherty, Max, et al.
Publicado: (2016)

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

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

A calcium message for Niemann-Pick type C
por: Cologna, Stephanie M.
Publicado: (2019)

Treatment trials in Niemann-Pick type C disease
por: Sitarska, Dominika, et al.
Publicado: (2021)

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)

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)

Fbxo2 mediates clearance of damaged lysosomes and modifies neurodegeneration in the Niemann-Pick C brain
por: Liu, Elaine A., et al.
Publicado: (2020)

TPC2 rescues lysosomal storage in mucolipidosis type IV, Niemann–Pick type C1, and Batten disease
por: Scotto Rosato, Anna, et al.
Publicado: (2022)

Niemann Pick C disease: lessons from zebrafish development
Publicado: (2011)

Intestinal and Hepatic Niemann-Pick C1-Like 1
por: Park, Sung-Woo
Publicado: (2013)

Efficacy and ototoxicity of different cyclodextrins in Niemann–Pick C disease
por: Davidson, Cristin D., et al.
Publicado: (2016)

Retinal axonal degeneration in Niemann–Pick type C disease
por: Havla, Joachim, et al.
Publicado: (2020)

Estimated prevalence of Niemann–Pick type C disease in Quebec
por: Labrecque, Marjorie, et al.
Publicado: (2021)

Spectrum of Movement Disorders in Niemann-Pick Disease Type C
por: Devaraj, Rashmi, et al.
Publicado: (2022)

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

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

Adenovirus RID-α activates an autonomous cholesterol regulatory mechanism that rescues defects linked to Niemann-Pick disease type C
por: Cianciola, Nicholas L., et al.
Publicado: (2009)

Niemann-Pick Disease: A Case Report and Literature Review
por: Vélez Pinos, Paola Jacqueline, et al.
Publicado: (2023)

Photomyogenic response in Niemann–Pick type C: a case report
por: Bour, A., et al.
Publicado: (2010)

Niemann–Pick disease type C: introduction and main clinical features
por: Burlina, A.
Publicado: (2014)

Genetic and laboratory diagnostic approach in Niemann Pick disease type C
por: McKay Bounford, K., et al.
Publicado: (2014)

Cannot write session to /tmp/vufind_sessions/sess_n13f22qrk11ja5l7t0mmkki3q3