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1
المؤلفون: Sri Harsha Meghadri, Beatriz Martinez-Delgado, Lena Ostermann, Gema Gomez-Mariano, Sara Perez-Luz, Srinu Tumpara, Sabine Wrenger, David S. DeLuca, Ulrich A. Maus, Tobias Welte, Sabina Janciauskiene
المساهمون: Instituto de Salud Carlos III
المصدر: International Journal of Molecular Sciences; Volume 23; Issue 18; Pages: 10425
مصطلحات موضوعية: Serine Proteinase Inhibitors, Data analysis, Gene Expression, liver, SERPINA, mice, gene knockout, RNA sequencing, transcriptomics, metabolism, single-cell, alpha1-antitrypsin, protein misfolding, data analysis, Gene knockout, Catalysis, Inorganic Chemistry, Mice, alpha 1-Antitrypsin Deficiency, Animals, Humans, RNA, Small Interfering, Physical and Theoretical Chemistry, Transcriptomics, Molecular Biology, Spectroscopy, Single-cell, Organic Chemistry, General Medicine, Computer Science Applications, Mice, Inbred C57BL, Metabolism, Cholesterol, Liver, alpha 1-Antitrypsin, Alpha1-antitrypsin, Metabolic Networks and Pathways, Protein misfolding
وصف الملف: application/pdf
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2دورية أكاديمية
المصدر: PLoS ONE; 8/1/2018, Vol. 13 Issue 8, p1-18, 18p
مصطلحات موضوعية: ALPHA 1-antitrypsin, URSODEOXYCHOLIC acid, AUTOPHAGY, LIVER cells, GENE transfection
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3
المؤلفون: Shana Gregory, Elena Montauti, Deyu Fang, Chuan He, Bryan T. Harada, Juncheng Wei, Yanan Xu, Nikita Mani, Donna D. Zhang, Beixue Gao, Richard M. Green, Samuel E. Weinberg, Dan Lu, Shuvam Chaudhuri, Ruihua Ma
المصدر: Mol Cell
مصطلحات موضوعية: Untranslated region, Ubiquitin-Protein Ligases, Apoptosis, Mice, SCID, Endoplasmic-reticulum-associated protein degradation, Endoplasmic Reticulum, Article, Mice, Ubiquitin, Mice, Inbred NOD, alpha 1-Antitrypsin Deficiency, Animals, Humans, Molecular Biology, Mice, Knockout, biology, Endoplasmic reticulum, MRNA modification, Adenine, Liver Diseases, Ubiquitination, Cell Biology, Endoplasmic Reticulum-Associated Degradation, Hep G2 Cells, Methyltransferases, Endoplasmic Reticulum Stress, Cell biology, Mice, Inbred C57BL, Disease Models, Animal, HEK293 Cells, Proteotoxicity, Liver, alpha 1-Antitrypsin, Proteolysis, biology.protein, Unfolded protein response, NIH 3T3 Cells, Transcription Factor CHOP
URL الوصول: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::e499afc30c713e8f2c0e70f467129551
https://europepmc.org/articles/PMC8751812/ -
4
المؤلفون: Francesco Callea, Hilda Angélica Martínez-Becerril, Magdalena Guerra-Crespo, Jerónimo F Garrido-Figueroa, Adriana Hernández-López, Marcia Hiriart, Rubén Argüero-Sánchez, Luis D. Bernal-Conde, Rodrigo Ramos-Acevedo, Francisco J Padilla-Godínez
المصدر: International Journal of Molecular Sciences, Vol 22, Iss 12467, p 12467 (2021)
International Journal of Molecular Sciencesمصطلحات موضوعية: Protein Folding, Parkinson's disease, alpha-synuclein, hereditary hypofibrinogenemia with hepatic storage, Cellular homeostasis, endoplasmic reticulum storage disease, Review, Protein aggregation, Endoplasmic Reticulum, chemistry.chemical_compound, protein misfolding, Biology (General), Spectroscopy, Parkinson Disease, General Medicine, Afibrinogenemia, Computer Science Applications, Cell biology, Chemistry, Neuroprotective Agents, Liver, QH301-705.5, alpha-1-antitrypsin, Biology, Catalysis, protein aggregation, Inorganic Chemistry, Protein Aggregates, alpha 1-Antitrypsin Deficiency, medicine, Autophagy, Animals, Humans, Protease Inhibitors, Physical and Theoretical Chemistry, Molecular Biology, QD1-999, Alpha-synuclein, Coagulants, Endoplasmic reticulum, Organic Chemistry, alpha-1-antitrypsin deficiency, Fibrinogen, medicine.disease, Proteostasis, chemistry, Gene Expression Regulation, alpha 1-Antitrypsin, Unfolded protein response, Unfolded Protein Response, Parkinson’s disease
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المؤلفون: Beatriz Martinez-Delgado, Selene Martínez, C. Jimenez, Gema Gomez-Mariano, Manuel Posada, M. I. Martínez, Meritxell Huch, Sara Monzón, Ignacio Pérez de Castro, Nerea Matamala, Iago Justo, Isabel Cuesta, Alberto Marcacuzco, Cristina Garfia, Sabina Janciauskiene
المساهمون: Martínez-Delgado, Beatriz [0000-0001-6834-350X], Apollo - University of Cambridge Repository, Instituto de Salud Carlos III
المصدر: Hepatology International
Repisalud
Instituto de Salud Carlos III (ISCIII)مصطلحات موضوعية: Liver Cirrhosis, 0301 basic medicine, congenital, hereditary, and neonatal diseases and abnormalities, Apolipoprotein B, Oncostatin M, 03 medical and health sciences, Liver disease, 0302 clinical medicine, alpha 1-Antitrypsin Deficiency, Gene expression, medicine, Organoid, Humans, Gene, Alpha 1-antitrypsin deficiency, Hepatology, biology, business.industry, Models, Theoretical, medicine.disease, Molecular biology, Organoids, 030104 developmental biology, medicine.anatomical_structure, Liver, Alpha-1 antitrypsin deficiency, alpha 1-Antitrypsin, 030220 oncology & carcinogenesis, Hepatocyte, biology.protein, Original Article, SERPINA1, RNA-seq, business
وصف الملف: text/xml; application/zip; application/pdf
URL الوصول: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::5af17ea510c459affca593750154fe59
https://doi.org/10.1007/s12072-019-10007-y -
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المؤلفون: Isabella Giovannoni, Francesco Callea, Paola Francalanci
المصدر: International Journal of Molecular Sciences
International Journal of Molecular Sciences, Vol 22, Iss 5778, p 5778 (2021)مصطلحات موضوعية: 0301 basic medicine, medicine.medical_specialty, congenital, hereditary, and neonatal diseases and abnormalities, QH301-705.5, Kupffer Cells, alpha-1-antitrypsin, Review, Fibrinogen, Endoplasmic Reticulum, Catalysis, Inorganic Chemistry, 03 medical and health sciences, 0302 clinical medicine, Microscopy, Electron, Transmission, Internal medicine, alpha 1-Antitrypsin Deficiency, medicine, Humans, Biology (General), Physical and Theoretical Chemistry, Hereditary hypofibrinogenemia, QD1-999, Molecular Biology, Gene, Spectroscopy, Chemistry, Endoplasmic reticulum, Organic Chemistry, Acute-phase protein, General Medicine, deficiency, Afibrinogenemia, Computer Science Applications, extrahepatic, 030104 developmental biology, Endocrinology, Liver, 030220 oncology & carcinogenesis, alpha 1-Antitrypsin, Mutation, Hepatocytes, fibrinogen, hepatic, manifestation, medicine.drug
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7دورية أكاديمية
المؤلفون: Khodayari, Nazli, Wang, Rejean liqun, Marek, George, Krotova, Karina, Kirst, Mariana, Liu, Chen, Rouhani, Farshid, Brantly, Mark
المصدر: PLoS ONE; 3/16/2017, Vol. 12 Issue 3, p1-19, 19p
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8
المؤلفون: Edwin K. Silverman, Phil Grayeski, Brian D. Hobbs, Craig P. Hersh, Peter J. Castaldi, Aaztli Coria, Silvia B. V. Ramos, Zhonghui Xu, Victor E Ortega, Michael H. Cho, Lela Lackey, Alain Laederach, John Platig, Vijay Shankar, Auyon J. Ghosh, Luke Hatfield
المصدر: PLoS Genetics, Vol 17, Iss 11, p e1009912 (2021)
PLoS Geneticsمصطلحات موضوعية: Untranslated region, Cancer Research, Polyadenylation, Pulmonology, Molecular biology, T-Lymphocytes, Gene Expression, RNA-binding protein, RNA-binding proteins, QH426-470, Biochemistry, Pulmonary Disease, Chronic Obstructive, Sequencing techniques, Untranslated Regions, Medicine and Health Sciences, RNA-Seq, RNA structure, Lung, Genetics (clinical), Messenger RNA, RNA sequencing, Stop codon, Nucleic acids, Liver, Codon, Terminator, Single-Cell Analysis, Research Article, 3' Utr, Chronic Obstructive Pulmonary Disease, Proteinase Inhibitory Proteins, Secretory, Biology, Cell Line, Genetics, Humans, Binding site, Ecology, Evolution, Behavior and Systematics, Reporter gene, Biology and life sciences, Three prime untranslated region, Macrophages, Proteins, Research and analysis methods, Macromolecular structure analysis, Molecular biology techniques, Gene Expression Regulation, alpha 1-Antitrypsin, Hepatocytes, RNA, Protein Translation
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المؤلفون: Karen McQuillan, Andrew A. Wilson, Danielle M Dunlea, Vipatsorn Shutchaidat, Joanne Keenan, Mark R. Wormald, Noel G. McElvaney, Michael Henry, Oliver J. McElvaney, Tomás P. Carroll, Paula Meleady, Emer P. Reeves, Prithvi Reddy Akepati, Darrell N. Kotton, Ciara A. O’Dwyer, Radka Saldova, Pauline M. Rudd, Derek C. Liberti
المصدر: The Journal of Immunology. 202:2240-2253
مصطلحات موضوعية: Adult, Male, congenital, hereditary, and neonatal diseases and abnormalities, Leukotriene B4, Induced Pluripotent Stem Cells, Immunology, Nonsense mutation, Immune Regulation, 03 medical and health sciences, chemistry.chemical_compound, 0302 clinical medicine, alpha 1-Antitrypsin Deficiency, Humans, Immunology and Allergy, Medicine, Alleles, chemistry.chemical_classification, Messenger RNA, Leukotriene, biology, business.industry, Acute-phase protein, Molecular biology, Ataluren, Liver, chemistry, Codon, Nonsense, alpha 1-Antitrypsin, Neutrophil elastase, biology.protein, Female, Glycoprotein, business, 030215 immunology
URL الوصول: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::6d2c5a22e237ceedbb981d177ff565e8
https://doi.org/10.4049/jimmunol.1801045 -
10
المؤلفون: Charles F Albright, Haiyan Jiang, Minerva E Sanchez, Eugenio Marco, Jeffrey Teckman, Keith Blomenkamp, Shen Shen, Erik M Corcoran, Clifford Yudkoff, David Bumcrot
المصدر: Human Gene Therapy. 29:861-873
مصطلحات موضوعية: 0301 basic medicine, Genetically modified mouse, congenital, hereditary, and neonatal diseases and abnormalities, Cirrhosis, Genetic enhancement, Genetic Vectors, Mice, Transgenic, Biology, medicine.disease_cause, Mice, 03 medical and health sciences, Exon, Liver disease, alpha 1-Antitrypsin Deficiency, Genetics, medicine, Animals, Humans, Molecular Biology, Gene, Alleles, Gene Editing, Mutation, Alpha 1-antitrypsin deficiency, Homozygote, Genetic Therapy, Dependovirus, medicine.disease, Molecular biology, Disease Models, Animal, 030104 developmental biology, Liver, alpha 1-Antitrypsin, Hepatocytes, Molecular Medicine, CRISPR-Cas Systems
URL الوصول: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::9fbb6898f93b170875e8aa11288389ad
https://doi.org/10.1089/hum.2017.227
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