Cargando…

ATP7B gene therapy of autologous reprogrammed hepatocytes alleviates copper accumulation in a mouse model of Wilson’s disease

BACKGROUND AND AIMS: Wilson’s disease (WD) is a rare hereditary disorder due to ATP7B gene mutation, causing pathologic copper storage mainly in the liver and neurological systems. Hepatocyte transplantation showed therapeutic potential; however, this strategy is often hindered by a shortage of qual...

Descripción completa

Detalles Bibliográficos
Autores principales:	Cai, Hongxia, Cheng, Xing, Wang, Xiao‐Ping
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	John Wiley and Sons Inc. 2022
Materias:	Original Articles
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9790736/ https://www.ncbi.nlm.nih.gov/pubmed/35340061 http://dx.doi.org/10.1002/hep.32484

Ejemplares similares

Systemic deletion of Atp7b modifies the hepatocytes’ response to copper overload in the mouse models of Wilson disease
por: Muchenditsi, Abigael, et al.
Publicado: (2021)

Structure of the Wilson disease copper transporter ATP7B
por: Bitter, Ryan M., et al.
Publicado: (2022)

Analysis of Wilson disease mutations in copper binding domain of ATP7B gene
por: Gul, Bushra, et al.
Publicado: (2022)

Wilson Disease Protein ATP7B Utilizes Lysosomal Exocytosis to Maintain Copper Homeostasis
por: Polishchuk, Elena V., et al.
Publicado: (2014)

Association of the canine ATP7A and ATP7B with hepatic copper accumulation in Dobermann dogs
por: Wu, Xiaoyan, et al.
Publicado: (2019)

Retinoids rescue ceruloplasmin secretion and alleviate oxidative stress in Wilson’s disease-specific hepatocytes
por: Song, Dan, et al.
Publicado: (2022)

Characterization of the most frequent ATP7B mutation causing Wilson disease in hepatocytes from patient induced pluripotent stem cells
por: Parisi, Silvia, et al.
Publicado: (2018)

ATP7B-Deficient Hepatocytes Reveal the Importance of Protein Misfolding Induced at Low Copper Concentration
por: Charbonnier, Peggy, et al.
Publicado: (2022)

The Structure of Metal Binding Domain 1 of the Copper Transporter ATP7B Reveals Mechanism of a Singular Wilson Disease Mutation
por: Yu, Corey H., et al.
Publicado: (2018)

Author Correction: Characterization of the most frequent ATP7B mutation causing Wilson disease in hepatocytes from patient induced pluripotent stem cells
por: Parisi, Silvia, et al.
Publicado: (2020)

Prevalence of ATP7B Gene Mutations in Iranian Patients With Wilson Disease
por: Zali, Narges, et al.
Publicado: (2011)

Functional Characterization of Novel ATP7B Variants for Diagnosis of Wilson Disease
por: Guttmann, Sarah, et al.
Publicado: (2018)

Hepatic copper accumulation in a young cat with familial variations in the ATP7B gene
por: Asada, Hajime, et al.
Publicado: (2018)

Monitoring of Copper in Wilson Disease
por: Gromadzka, Grażyna, et al.
Publicado: (2023)

Wilson Disease: Intersecting DNA Methylation and Histone Acetylation Regulation of Gene Expression in a Mouse Model of Hepatic Copper Accumulation
por: Sarode, Gaurav V., et al.
Publicado: (2021)

ATP7B R778L mutant hepatocytes resist copper toxicity by activating autophagy and inhibiting necroptosis
por: Tang, Shan, et al.
Publicado: (2023)

Novel mutations found in the ATP7B gene in Chinese patients with Wilson's disease
por: Qian, Zhiling, et al.
Publicado: (2019)

Genetic analysis of ATP7B in 102 south Indian families with Wilson disease
por: Singh, Nivedita, et al.
Publicado: (2019)

A Novel Mutation of ATP7B Gene in a Case of Wilson Disease
por: Kahraman, Cigdem Yuce, et al.
Publicado: (2021)

A case of Wilson disease with the ATP7B mutation presenting movement disorders
por: Van Nguyen, Huong, et al.
Publicado: (2021)

Mutation spectrum of ATP7B gene in pediatric patients with Wilson disease in Vietnam
por: Huong, Nguyen Thi Mai, et al.
Publicado: (2022)

New ATP7B Gene Mutation in a Brazilian Patient with Wilson Disease
por: Barros de Oliveira Sá, Marcus Villander, et al.
Publicado: (2022)

Identification of Lncrna-Mrna Networks in Hepg2 Cells upon ATP7B Knockout and Copper Accumulation
por: Yan, Yan, et al.
Publicado: (2023)

Intragenic Deletions in ATP7B as an Unusual Molecular Genetics Mechanism of Wilson’s Disease Pathogenesis
por: Todorov, Theodor, et al.
Publicado: (2016)

Three novel mutations in the ATP7B gene of unrelated Vietnamese patients with Wilson disease
por: Huong, Nguyen Thi Mai, et al.
Publicado: (2018)

Genetic variation spectrum in ATP7B gene identified in Latvian patients with Wilson disease
por: Zarina, Agnese, et al.
Publicado: (2017)

Identification of mutations in the ATP7B gene in 14 Wilson disease children: Case series
por: Wang, Jiuxiang, et al.
Publicado: (2021)

Truncating mutations in the Wilson disease gene ATP7B are associated with very low serum ceruloplasmin oxidase activity and an early onset of Wilson disease
por: Merle, Uta, et al.
Publicado: (2010)

Role of the P-Type ATPases, ATP7A and ATP7B in brain copper homeostasis
por: Telianidis, Jonathon, et al.
Publicado: (2013)

Bromide alleviates fatty acid‐induced lipid accumulation in mouse primary hepatocytes through the activation of PPARα signals
por: Shi, Yujie, et al.
Publicado: (2019)

Molecular mechanism of copper transport in Wilson disease.
por: Fatemi, Negah, et al.
Publicado: (2002)

The Paradox of Copper Deficiency in Wilson's Disease Patients
por: Iorio, Raffaele, et al.
Publicado: (2023)

Novel mutations in ATP7B in Chinese patients with Wilson's disease and identification of kidney disorder of thinning of the glomerular basement membrane
por: Xu, Hongliang, et al.
Publicado: (2023)

Genetic, metabolic and cellular factors influencing intracellular localization of the Wilson disease protein, ATP7B
por: Gupta, Arnab, et al.
Publicado: (2014)

Early-onset Wilson disease caused by ATP7B exon skipping associated with intronic variant
por: Koboldt, Daniel C., et al.
Publicado: (2020)

Investigation of the Wilson gene ATP7B transcriptional start site and the effect of core promoter alterations
por: Höflich, Clemens, et al.
Publicado: (2021)

Mutation analysis of the ATP7B gene and genotype–phenotype correlation in Chinese patients with Wilson disease
por: Li, Mingming, et al.
Publicado: (2021)

Laboratory and clinical evaluation of a microarray for the detection of ATP7B mutations in Wilson disease in China
por: Jia, Siyu, et al.
Publicado: (2022)

Analyzing the Therapeutic Efficacy of Bis-Choline-Tetrathiomolybdate in the Atp7b(−/−) Copper Overload Mouse Model
por: Kim, Philipp, et al.
Publicado: (2021)

DPM-1001 decreased copper levels and ameliorated deficits in a mouse model of Wilson's disease
por: Krishnan, Navasona, et al.
Publicado: (2018)

Cannot write session to /tmp/vufind_sessions/sess_d0puegibq73rvo8lo5hu9n907m