Cargando…

A Novel Small Molecule Inhibits Intrahepatocellular Accumulation of Z-Variant Alpha 1-Antitrypsin In Vitro and In Vivo

Alpha 1-antitrypsin deficiency (AATD) is the most common genetic cause of liver disease in children and is associated with early-onset chronic liver disease in adults. AATD associated liver injury is caused by hepatotoxic retention of polymerized mutant alpha 1-antitrypsin molecules within the endop...

Descripción completa

Detalles Bibliográficos
Autores principales:	Zhang, Xiaojuan, Pham, Kien, Li, Danmeng, Schutte, Ryan J., Gonzalo, David Hernandez, Zhang, Penghui, Oshins, Regina, Tan, Weihong, Brantly, Mark, Liu, Chen, Ostrov, David A.
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	MDPI 2019
Materias:	Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6953066/ https://www.ncbi.nlm.nih.gov/pubmed/31817705 http://dx.doi.org/10.3390/cells8121586

Ejemplares similares

The unfolded protein response to PI*Z alpha‐1 antitrypsin in human hepatocellular and murine models
por: Lu, Yuanqing, et al.
Publicado: (2022)

Alpha 1 Antitrypsin-Deficient Macrophages Have Impaired Efferocytosis of Apoptotic Neutrophils
por: Lee, Jungnam, et al.
Publicado: (2020)

Alpha-1 antitrypsin deficient individuals have circulating extracellular vesicles with profibrogenic cargo
por: Khodayari, Nazli, et al.
Publicado: (2020)

The Mechanism of Mitochondrial Injury in Alpha-1 Antitrypsin Deficiency Mediated Liver Disease
por: Khodayari, Nazli, et al.
Publicado: (2021)

Intrahepatic heteropolymerization of M and Z alpha-1-antitrypsin
por: Laffranchi, Mattia, et al.
Publicado: (2020)

Correlation of alpha-1 antitrypsin levels and exosome associated neutrophil elastase endothelial injury in subjects with SARS-CoV2 infection
por: Lascano, Jorge, et al.
Publicado: (2022)

Erdj3 Has an Essential Role for Z Variant Alpha‐1‐Antitrypsin Degradation
por: Khodayari, Nazli, et al.
Publicado: (2017)

Determining the temporal, dose, and composition effects of nutritional substrates in an in vitro model of intrahepatocellular triglyceride accumulation
por: Nagarajan, Shilpa R., et al.
Publicado: (2022)

Cigarette smoke exposed airway epithelial cell-derived EVs promote pro-inflammatory macrophage activation in alpha-1 antitrypsin deficiency
por: Khodayari, Nazli, et al.
Publicado: (2022)

Correction to: Cigarette smoke exposed airway epithelial cells-derived EVs promote pro-inflammatory macrophage activation in alpha-1 antitrypsin deficiency
por: Khodayari, Nazli, et al.
Publicado: (2023)

The Effect of Aerobic Exercise on Intrahepatocellular and Intramyocellular Lipids in Healthy Subjects
por: Egger, Andrea, et al.
Publicado: (2013)

SVIP regulates Z variant alpha-1 antitrypsin retro-translocation by inhibiting ubiquitin ligase gp78
por: Khodayari, Nazli, et al.
Publicado: (2017)

Discovery of an Inhibitor of Z-Alpha1 Antitrypsin Polymerization
por: Berthelier, Valerie, et al.
Publicado: (2015)

Is PiSS Alpha-1 Antitrypsin Deficiency Associated with Disease?
por: McGee, Dawn, et al.
Publicado: (2010)

Sexual dimorphism in relation to adipose tissue and intrahepatocellular lipid deposition in early infancy
por: Gale, C, et al.
Publicado: (2015)

Lung Inflammation in alpha-1-antitrypsin deficient individuals with normal lung function
por: Kokturk, Nurdan, et al.
Publicado: (2023)

Alpha-1-antitrypsin augmentation therapy in deficient individuals enrolled in the Alpha-1 Foundation DNA and Tissue Bank
por: Tonelli, Adriano R, et al.
Publicado: (2009)

Development of a risk score to increase detection of severe alpha-1 antitrypsin deficiency
por: Riley, E. Leonard, et al.
Publicado: (2023)

Alpha-1 Antitrypsin Attenuates Acute Lung Allograft Injury in a Rat Lung Transplant Model
por: Emtiazjoo, Amir M., et al.
Publicado: (2019)

Quantitative measurement of the histological features of alpha-1 antitrypsin deficiency-associated liver disease in biopsy specimens
por: Marek, George, et al.
Publicado: (2021)

Detection of alpha-1 antitrypsin deficiency: the past, present and future
por: Brantly, Mark, et al.
Publicado: (2020)

Role of Drosophila EDEMs in the degradation of the alpha-1-antitrypsin Z variant
por: JANG, BO-YUN, et al.
Publicado: (2015)

Alpha-1 antitrypsin deficiency impairs lung antibacterial immunity in mice
por: Ostermann, Lena, et al.
Publicado: (2021)

The role of bronchial epithelial cells in the pathogenesis of COPD in Z-alpha-1 antitrypsin deficiency
por: Pini, Laura, et al.
Publicado: (2014)

Risk factors for symptom onset in PI*Z alpha-1 antitrypsin deficiency
por: Mayer, Annyce S, et al.
Publicado: (2006)

Prevalence of PI*Z and PI*S alleles of alpha-1-antitrypsin deficiency in Finland
por: Häggblom, Jan, et al.
Publicado: (2015)

Heterozygosity of the Alpha 1‐Antitrypsin Pi*Z Allele and Risk of Liver Disease
por: Hakim, Aaron, et al.
Publicado: (2021)

Neutrophil elastase promotes macrophage cell adhesion and cytokine production through the integrin-Src kinases pathway
por: Krotova, Karina, et al.
Publicado: (2020)

Mineralization of alpha-1-antitrypsin inclusion bodies in Mmalton alpha-1-antitrypsin deficiency
por: Callea, Francesco, et al.
Publicado: (2018)

Alpha-1 Antitrypsin Z Variant (AAT PI*Z) as a Risk Factor for Intrahepatic Cholestasis of Pregnancy
por: Kosinski, Przemyslaw, et al.
Publicado: (2021)

Heterozygosity for the alpha‐1‐antitrypsin Z allele in cirrhosis is associated with more advanced disease
por: Schaefer, Benedikt, et al.
Publicado: (2018)

Pharmacokinetic comparability of Prolastin(®)-C to Prolastin(® )in alpha(1)-antitrypsin deficiency: a randomized study
por: Stocks, James M, et al.
Publicado: (2010)

Alpha-1 antitrypsin protein and gene therapies decrease autoimmunity and delay arthritis development in mouse model
por: Grimstein, Christian, et al.
Publicado: (2011)

Gene therapy for alpha 1-antitrypsin deficiency with an oxidant-resistant human alpha 1-antitrypsin
por: Sosulski, Meredith L., et al.
Publicado: (2020)

Alpha-1 Antitrypsin as a Therapeutic Agent for Conditions not Associated with Alpha-1 Antitrypsin Deficiency
por: Wanner, Adam
Publicado: (2015)

Appropriateness of Newborn Screening for α1-Antitrypsin Deficiency
por: Teckman, Jeffrey, et al.
Publicado: (2014)

Quantitation of circulating wild-type alpha-1-antitrypsin in heterozygous carriers of the S and Z deficiency alleles
por: Donato, L. J., et al.
Publicado: (2015)

Safety and efficacy of alpha-1-antitrypsin augmentation therapy in the treatment of patients with alpha-1-antitrypsin deficiency
por: Petrache, Irina, et al.
Publicado: (2009)

Alpha-1-Antitrypsin Deficiency, the Serpinopathies and Conformational Disease
por: Parmar, Jas S, et al.
Publicado: (2000)

Alpha 1 antitrypsin to treat lung disease in alpha 1 antitrypsin deficiency: recent developments and clinical implications
por: Chapman, Kenneth R, et al.
Publicado: (2018)

Cannot write session to /tmp/vufind_sessions/sess_9la464sieqmbsd0288aal9bdit