المؤلفون: Jie Li, Francesca Moretti, Tunda Hidvegi, Sanja Sviben, James A.J. Fitzpatrick, Hemalatha Sundaramoorthi, Stephen C. Pak, Gary A. Silverman, Britta Knapp, Ireos Filipuzzi, John Alford, John Reece-Hoyes, Florian Nigsch, Leon O. Murphy, Beat Nyfeler, David H. Perlmutter

المصدر: Cellular and Molecular Gastroenterology and Hepatology, Vol 17, Iss 6, Pp 1007-1024 (2024)

مصطلحات موضوعية: Autophagy, Proteasome, Aggregation-Prone Proteins, α1-Antitrypsin Deficiency, Liver Disease, Diseases of the digestive system. Gastroenterology, RC799-869

وصف الملف: electronic resource

Relation: http://www.sciencedirect.com/science/article/pii/S2352345X24000341; https://doaj.org/toc/2352-345X

URL الوصول: https://doaj.org/article/732b690fbbd84589915ab3fbdd2ec291

المؤلفون: Yan Wang, Murat C Cobanoglu, Jie Li, Tunda Hidvegi, Pamela Hale, Michael Ewing, Andrew S Chu, Zhenwei Gong, Radhika Muzumdar, Stephen C Pak, Gary A Silverman, Ivet Bahar, David H Perlmutter

المصدر: PLoS ONE, Vol 14, Iss 1, p e0209748 (2019)

مصطلحات موضوعية: Medicine, Science

وصف الملف: electronic resource

Relation: https://doaj.org/toc/1932-6203

URL الوصول: https://doaj.org/article/15ce59d7c3b44813913242c9f529345a

المؤلفون: Jie Li, Stephen C Pak, Linda P O'Reilly, Joshua A Benson, Yan Wang, Tunda Hidvegi, Pamela Hale, Christine Dippold, Michael Ewing, Gary A Silverman, David H Perlmutter

المصدر: PLoS ONE, Vol 9, Iss 1, p e87260 (2014)

مصطلحات موضوعية: Medicine, Science

وصف الملف: electronic resource

Relation: http://europepmc.org/articles/PMC3909079?pdf=render; https://doaj.org/toc/1932-6203

URL الوصول: https://doaj.org/article/c378e72431a64ece8d6c33c6f47ce71c

المؤلفون: David A. Rudnick, Jiansheng Huang, Tunda Hidvegi, Andrew S. Chu, Pamela Hale, Admire Munanairi, Dennis J. Dietzen, Paul F. Cliften, Eric Tycksen, Andrew J. Lutkewitte, Brian N. Finck, Stephen C. Pak, Gary A. Silverman, David H. Perlmutter

المصدر: Gastroenterology. 163(1)

مصطلحات موضوعية: Liver Cirrhosis, Mammals, Mice, Hepatology, Liver, alpha 1-Antitrypsin Deficiency, Mutation, Gastroenterology, Animals, Insulin, Mice, Transgenic, Peroxisome Proliferator-Activated Receptor Gamma Coactivator 1-alpha, Signal Transduction

URL الوصول: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::fa4b6cb469d84421c34ab22fe9ce0a95
https://pubmed.ncbi.nlm.nih.gov/35500618

المؤلفون: Tunda Hidvegi, Patrick Araya, Michael Ewing, Donna B. Stolz, David H. Perlmutter, Amitava Mukherjee

مصطلحات موضوعية: 0301 basic medicine, Protein Folding, Transgene, Down-Regulation, Mice, Transgenic, Biology, medicine.disease_cause, Article, Transcriptome, 03 medical and health sciences, Mice, 0302 clinical medicine, Downregulation and upregulation, Fibrosis, alpha 1-Antitrypsin Deficiency, medicine, Autophagy, Animals, Humans, Molecular Biology, Early Growth Response Protein 1, NF-kappa B, Cell Biology, medicine.disease, Cell biology, Mice, Inbred C57BL, 030104 developmental biology, Proteostasis, Liver, 030220 oncology & carcinogenesis, alpha 1-Antitrypsin, Signal transduction, Carcinogenesis, HeLa Cells

URL الوصول: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::5578803e2e9f11553c4893d0e0445a58
https://europepmc.org/articles/PMC6370812/

المؤلفون: Tunda Hidvegi, Ivet Bahar, Stephen C. Pak, Jie Li, David H. Perlmutter, Pamela Hale, Murat Can Cobanoglu, Andrew S. Chu, Radhika Muzumdar, Gary A. Silverman, Yan Wang, Michael Ewing, Zhenwei Gong

المصدر: PLoS ONE
PLoS ONE, Vol 14, Iss 1, p e0209748 (2019)

مصطلحات موضوعية: Liver Cirrhosis, 0301 basic medicine, Nematoda, Physiology, Inflammasomes, medicine.medical_treatment, Pharmacology, Biochemistry, Glibenclamide, Mice, Endocrinology, 0302 clinical medicine, RNA interference, Insulin Secretion, Drug Discovery, Glyburide, Medicine and Health Sciences, Insulin, Small interfering RNAs, Immune System Proteins, Multidisciplinary, Cell Death, Drug discovery, Liver Diseases, Eukaryota, Animal Models, 3. Good health, Nucleic acids, Experimental Organism Systems, Liver, Cell Processes, Caenorhabditis Elegans, 030220 oncology & carcinogenesis, Medicine, RNA Interference, Intracellular, Research Article, medicine.drug, Science, Autophagic Cell Death, Transgene, Immunology, Mouse Models, Mice, Transgenic, Gastroenterology and Hepatology, Research and Analysis Methods, Cell Line, 03 medical and health sciences, Model Organisms, In vivo, alpha 1-Antitrypsin Deficiency, Genetics, Autophagy, medicine, Animals, Humans, Non-coding RNA, Caenorhabditis elegans Proteins, Diabetic Endocrinology, Endocrine Physiology, Organisms, Biology and Life Sciences, Proteins, Cell Biology, Invertebrates, Hormones, Gene regulation, Disease Models, Animal, 030104 developmental biology, alpha 1-Antitrypsin, Animal Studies, Caenorhabditis, RNA, Gene expression

URL الوصول: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::32ee8e5dd7dd1df0f732af39a74aafdf
https://doi.org/10.1371/journal.pone.0209748

المؤلفون: David H. Perlmutter, Jianqiang Ding, Jayanta Roy-Chowdhury, Govardhana Rao Yannam, Chandan Guha, Namita Roy-Chowdhury, Hesham Basma, Tunda Hidvegi, Yesim Avsar, Ira J. Fox, Ronald J. Wong, Stephen I. Rennard

المصدر: Journal of Clinical Investigation. 121:1930-1934

مصطلحات موضوعية: congenital, hereditary, and neonatal diseases and abnormalities, Mice, Transgenic, Mice, Liver disease, Mutant protein, Fibrosis, Albumins, medicine, Animals, Humans, Transplantation, Homologous, Transgenes, Liver injury, biology, Brief Report, Wild type, Alanine Transaminase, Bilirubin, General Medicine, medicine.disease, Molecular biology, Mice, Inbred C57BL, medicine.anatomical_structure, Liver, alpha 1-Antitrypsin, Neutrophil elastase, Hepatocyte, Mutation, Immunology, Hepatocytes, biology.protein, Hepatocyte growth factor, medicine.drug

URL الوصول: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::9af1c949917a27a618f5b60e08fcd733
https://doi.org/10.1172/jci45260

المؤلفون: Michael Ewing, Caroline Beckett, Christine S. Dippold, Christina Goldbach, Simon C. Watkins, Carolyn Kemp, Nicholas Maurice, Tunda Hidvegi, Amitava Mukherjee, George K. Michalopoulos, Pamela Hale, David H. Perlmutter

المصدر: Science. 329:229-232

مصطلحات موضوعية: Liver Cirrhosis, Proteasome Endopeptidase Complex, Protein Folding, medicine.medical_specialty, Transgene, Mutant, Mice, Transgenic, Biology, Endoplasmic Reticulum, medicine.disease_cause, Cell Line, Mice, Phagosomes, alpha 1-Antitrypsin Deficiency, Internal medicine, Autophagy, medicine, Animals, Humans, Mutation, Multidisciplinary, Point mutation, Disease Models, Animal, Carbamazepine, Phenotype, Endocrinology, Liver, Solubility, Mechanism of action, alpha 1-Antitrypsin, Cancer research, Mutant Proteins, medicine.symptom, Hepatic fibrosis, Carcinogenesis, HeLa Cells

URL الوصول: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::97720ded03580b55a6bdf95a82f0a277
https://doi.org/10.1126/science.1190354

المؤلفون: Pamela Hale, Michael Ewing, Caroline Beckett, David H. Perlmutter, Tunda Hidvegi, Karoly Mirnics

المصدر: Journal of Biological Chemistry. 282:27769-27780

مصطلحات موضوعية: Protein Denaturation, Protein Conformation, Transgene, Mutant, Mice, Transgenic, Biology, Endoplasmic Reticulum, Models, Biological, Biochemistry, Mice, chemistry.chemical_compound, Mutant protein, Gene expression, Animals, Humans, Molecular Biology, Endoplasmic reticulum, Cell Biology, Tunicamycin, Cell biology, Liver, chemistry, alpha 1-Antitrypsin, Mutation, Hepatocytes, Unfolded protein response, Signal transduction, RGS Proteins, HeLa Cells, Signal Transduction

URL الوصول: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::eaddecdbb89148454439b017c18575a4
https://doi.org/10.1074/jbc.m704330200

المؤلفون: Samuel A. Yousem, Nunzia Pastore, Pamela Hale, Steven D. Shapiro, Patrizia Annunziata, Michael Ewing, Nicola Brunetti-Pierri, Stephen C. Pak, Naftali Kaminski, Donna B. Stolz, Jieru Wang, Joseph M. Pilewski, Adriana S. Leme, Evelyn Akpadock Garchar, A. McGarry Houghton, David H. Perlmutter, Gary A. Silverman, Tunda Hidvegi, Houming Cai, John F. Alcorn

المساهمون: Hidvegi, Tunda, Stolz, Donna B, Alcorn, John F, Yousem, Samuel A, Wang, Jieru, Leme, Adriana S, Houghton, A. McGarry, Hale, Pamela, Ewing, Michael, Cai, Houming, Garchar, Evelyn Akpadock, Pastore, Nunzia, Annunziata, Patrizia, Kaminski, Naftali, Pilewski, Joseph, Shapiro, Steven D, Pak, Stephen C, Silverman, Gary A, BRUNETTI PIERRI, Nicola, Perlmutter, David H.

مصطلحات موضوعية: pulmonary fibrosi, autophagy, Genetic enhancement, misfolded α1antitrypsin, macromolecular substances, Biology, Biochemistry, Mice, alpha 1-Antitrypsin Deficiency, Pulmonary fibrosis, medicine, Animals, Respiratory system, protein misfolding, chronic obstructive pulmonary disease (COPD), Molecular Biology, Lung, Autophagy, α1antitrypsin deficiency, Molecular Bases of Disease, Epithelial Cells, Cell Biology, Genetic Therapy, respiratory system, proteostasi, medicine.disease, Surfactant protein A, respiratory tract diseases, Disease Models, Animal, Proteostasis, medicine.anatomical_structure, Immunology, Cancer research, TFEB

URL الوصول: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::d6a3f2445bf8ce202154e7ce4dababe9
http://hdl.handle.net/11588/614053