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Protein Misfolding and the Serpinopathies

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Pages 15-20 | Received 22 Jan 2007, Accepted 06 Feb 2007, Published online: 01 Mar 2007

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Robert A Stockley. (2010) Emerging drugs for alpha-1-antitrypsin deficiency. Expert Opinion on Emerging Drugs 15:4, pages 685-694.
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Nicole R. Higgins, Jessie E. Greenslade, Josephine J. Wu, Elena Miranda, Giovanna Galliciotti & Mervyn J. Monteiro. (2021) Serpin neuropathology in the P497S UBQLN2 mouse model of ALS/FTD. Brain Pathology 31:5.
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Barbora Kascakova, Jan Kotal, Larissa Almeida Martins, Zuzana Berankova, Helena Langhansova, Eric Calvo, Joel A. Crossley, Petra Havlickova, Filip Dycka, Tatyana Prudnikova, Michal Kuty, Michail Kotsyfakis, Jindrich Chmelar & Ivana Kuta Smatanova. (2021) Structural and biochemical characterization of the novel serpin Iripin-5 from Ixodes ricinus . Acta Crystallographica Section D Structural Biology 77:9, pages 1183-1196.
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Mohamad Aman Jairajpuri & Shoyab Ansari. (2020) Using serpins cysteine protease cross-specificity to possibly trap SARS-CoV-2 Mpro with reactive center loop chimera. Clinical Science 134:17, pages 2235-2241.
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Manjumol Mathew, Charuvila T. Aravindakumar & Usha K. Aravind. (2020) Unravelling the fibrillation mechanism of ovalbumin in the presence of mercury at its isoelectric pH. RSC Advances 10:28, pages 16415-16421.
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Esra Karatas & Marion Bouchecareilh. (2020) Alpha 1-Antitrypsin Deficiency: A Disorder of Proteostasis-Mediated Protein Folding and Trafficking Pathways. International Journal of Molecular Sciences 21:4, pages 1493.
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Maddaloni V, Pepe N, Morano F, Lanzo M, Darco D & Atripaldi L. (2019) Alpha 1 antitrypsin deficiency: a rare allele in patients from south of Italy. Journal of Lung, Pulmonary & Respiratory Research 6:2, pages 32-35.
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Siv Meling, Kerstin Skovgaard, Kjetil Bårdsen, Peter Mikael Helweg Heegaard & Martha J. Ulvund. (2018) Expression of selected genes isolated from whole blood, liver and obex in lambs with experimental classical scrapie and healthy controls, showing a systemic innate immune response at the clinical end-stage. BMC Veterinary Research 14:1.
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Sergio B. Socias, Florencia González-Lizárraga, Cesar L. Avila, Cecilia Vera, Leonardo Acuña, Julia E. Sepulveda-Diaz, Elaine Del-Bel, Rita Raisman-Vozari & Rosana N. Chehin. (2018) Exploiting the therapeutic potential of ready-to-use drugs: Repurposing antibiotics against amyloid aggregation in neurodegenerative diseases. Progress in Neurobiology 162, pages 17-36.
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Krishna Sumanth Nallagangula, K.N. Shashidhar, V. Lakshmaiah & C. Muninarayana. (2017) Cirrhosis of liver: Interference of serpins in quantification of SERPINA4 – A preliminary study. Practical Laboratory Medicine 9, pages 53-57.
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Jindřich Chmelař, Jan Kotál, Helena Langhansová & Michail Kotsyfakis. (2017) Protease Inhibitors in Tick Saliva: The Role of Serpins and Cystatins in Tick-host-Pathogen Interaction. Frontiers in Cellular and Infection Microbiology 7.
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Prakash Baligar, Veena Kochat, Shailendra K. Arindkar, Zaffar Equbal, Snehashish Mukherjee, Swati Patel, Perumal Nagarajan, Sujata Mohanty, Jeffrey H. Teckman & Asok Mukhopadhyay. (2017) Bone marrow stem cell therapy partially ameliorates pathological consequences in livers of mice expressing mutant human α1‐antitrypsin. Hepatology 65:4, pages 1319-1335.
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Wayne J. Higgins, Gareth T. Grehan, Kieran J. Wynne & D. Margaret Worrall. (2017) SerpinI2 (pancpin) is an inhibitory serpin targeting pancreatic elastase and chymotrypsin. Biochimica et Biophysica Acta (BBA) - Proteins and Proteomics 1865:2, pages 195-200.
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S. Chakraborty, D.H. Perlmutter & Amitava Mukherjee. 2016. Encyclopedia of Cell Biology. Encyclopedia of Cell Biology 789 800 .
Antoine Péré-Brissaud, Xavier Blanchet, Didier Delourme, Patrick Pélissier, Lionel Forestier, Arnaud Delavaud, Nathalie Duprat, Brigitte Picard, Abderrahman Maftah & Laure Brémaud. (2015) Expression of SERPINA3s in cattle: focus on bovSERPINA3-7 reveals specific involvement in skeletal muscle. Open Biology 5:9, pages 150071.
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Daniel Elenius Madsen, Johannes Jakobsen Sidelmann, Daniel Biltoft, Jørgen Gram & Soren Hansen. (2015) C1-inhibitor polymers activate the FXII-dependent kallikrein–kinin system: Implication for a role in hereditary angioedema. Biochimica et Biophysica Acta (BBA) - General Subjects 1850:6, pages 1336-1342.
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Angela Schipanski, Felix Oberhauser, Melanie Neumann, Sascha Lange, Beata Szalay, Susanne Krasemann, Fred W. van Leeuwen, Giovanna Galliciotti & Markus Glatzel. (2014) The lectin OS-9 delivers mutant neuroserpin to endoplasmic reticulum associated degradation in familial encephalopathy with neuroserpin inclusion bodies. Neurobiology of Aging 35:10, pages 2394-2403.
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Philip J. Robinson & Cheryl A. Woolhead. 2013. FRET – Förster Resonance Energy Transfer. FRET – Förster Resonance Energy Transfer 357 396 .
X. Blanchet, A. Péré-Brissaud, N. Duprat, E. Pinault, D. Delourme, A. Ouali, C. Combet, A. Maftah, P. Pélissier & L. Brémaud. (2012) Mutagenesis of the bovSERPINA3-3 demonstrates the requirement of aspartate-371 for intermolecular interaction and formation of dimers. Protein Science 21:7, pages 977-986.
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Cheng-Jian Jiang, Zhen-Yu Hao, Rong Zeng, Pei-Hong Shen, Jun-Fang Li & Bo Wu. (2011) Characterization of a Novel Serine Protease Inhibitor Gene from a Marine Metagenome. Marine Drugs 9:9, pages 1487-1501.
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J.A. HUNTINGTON. (2011) Serpin structure, function and dysfunction. Journal of Thrombosis and Haemostasis 9, pages 26-34.
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Olivia S. Long, Sager J. Gosai, Joon Hyeok Kwak, Dale E. King, David H. Perlmutter, Gary A. Silverman & Stephen C. Pak. 2011. Biology of Serpins. Biology of Serpins 259 281 .
Shih-Hon Li & Daniel A. Lawrence. 2011. Serpin Structure and Evolution. Serpin Structure and Evolution 177 207 .
Masayuki Yamasaki, Timothy J. Sendall, Laura E. Harris, Giles M.W. Lewis & James A. Huntington. (2010) Loop-Sheet Mechanism of Serpin Polymerization Tested by Reactive Center Loop Mutations. Journal of Biological Chemistry 285:40, pages 30752-30758.
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Manni Luthra-Guptasarma & Purnananda Guptasarma. (2010) Metal-catalyzed proteolysis, conformational antigenicity, photosensitized oxidation, and electrical dysfunction explain the pathogenicity of protein aggregates. Medical Hypotheses 75:3, pages 294-298.
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Aaron Kerman, Hsueh-Ning Liu, Sidney Croul, Juan Bilbao, Ekaterina Rogaeva, Lorne Zinman, Janice Robertson & Avijit Chakrabartty. (2010) Amyotrophic lateral sclerosis is a non-amyloid disease in which extensive misfolding of SOD1 is unique to the familial form. Acta Neuropathologica 119:3, pages 335-344.
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Didier Belorgey, Peter Hägglöf, Maki Onda & David A. Lomas. (2010) pH-dependent stability of neuroserpin is mediated by histidines 119 and 138; Implications for the control of β-sheet A and polymerization. Protein Science 19:2, pages 220-228.
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