Ritratto di mariaelena.mirandabanos@uniroma1.it

CORSO Biologia Cellulare (Biotecnologie Agro-alimentari e Industriali)

 

Il corso inizia 2 ottobre 2023, ore 11:00, in aula II NEC (CU032).

 

Gli studenti sono pregati di aderire al corso tramite la prenotazione che troveranno nella pagina e-Learning del corso. 

 

Le lezioni si terranno in presenza.

 

Le slide delle lezioni sarano disponibili nel sito eLearning del corso:

https://elearning.uniroma1.it

 

 

 


 

 

CORSO Biologia Cellulare e Istologia (Scienze Biologiche)

 

Il corso inizia 2 ottobre 2023, ore 11:00, in aula II NEC (CU032).

 

Gli studenti sono pregati di aderire al corso tramite la prenotazione che troveranno nella pagina e-Learning del corso. 

 

Le lezioni si terranno in presenza.

 

Le slide delle lezioni sono disponibili nel sito eLearning del corso (piattaforma Moodle):

https://elearning.uniroma1.it

 

 

 


 

 

CORSO Biotecnologie Cellulari, modulo Cellule Animali (Scienze Biologiche)

 

Il corso inizia il 27 febbraio 2024, ore 11:00, in aula 218bis.

 

Gli studenti sono pregati di aderire al corso tramite la prenotazione che troveranno nella pagina e-Learning del corso. 

 

Le lezioni si terranno in presenza.

 

Le slide delle lezioni sono disponibili nel sito eLearning del corso (piattaforma Moodle):

https://elearning.uniroma1.it

 

 

 

Insegnamento Codice Anno Corso - Frequentare Bacheca
BIOLOGIA CELLULARE E ISTOLOGIA 1011792 2023/2024
BIOTECNOLOGIE CELLULARI 10600124 2023/2024
BIOLOGIA CELLULARE 1034846 2023/2024
BIOLOGIA CELLULARE 1034846 2022/2023
BIOTECNOLOGIE CELLULARI ANIMALI E SISTEMI DI COLTURA 1034879 2022/2023
BIOLOGIA CELLULARE 1034846 2021/2022
BIOTECNOLOGIE CELLULARI ANIMALI E SISTEMI DI COLTURA 1034879 2021/2022
BIOLOGIA CELLULARE 1034846 2020/2021
BIOTECNOLOGIE CELLULARI ANIMALI E SISTEMI DI COLTURA 1034879 2020/2021
BIOLOGIA CELLULARE 1034846 2019/2020
BIOTECNOLOGIE CELLULARI ANIMALI E SISTEMI DI COLTURA 1034879 2019/2020
BIOLOGIA CELLULARE 1034846 2018/2019
INTRACELLULAR TRAFFICKING 1051861 2017/2018
BIOLOGIA CELLULARE 1034846 2017/2018
BIOTECNOLOGIE CELLULARI ANIMALI E SISTEMI DI COLTURA 1034879 2017/2018
BASI MOLECOLARI DEL TRAFFICO INTRACELLULARE 1034857 2016/2017
BIOLOGIA CELLULARE 1034846 2016/2017

Su appuntamento tramite email. In modalità in presenza o tramite Google Meet.

CURRICULUM VITAE
November 2022

Personal information

Full name: Maria Elena Miranda Baños
Current position: Associate professor
Current address: Dipartimento di Biologia e Biotecnologie Charles Darwin ,
Sapienza Università di Roma
Piazzale Aldo Moro n. 5, 00185, Roma, Italia
Tel./Fax: +39 06 4991 2637/ +39 06 4991 2351
E-mail: mariaelena.mirandabanos@uniroma1.it

Education
- 10/1990-07/1995: Undergraduate student, University of Malaga (Spain).
- 07/1995: Degree in Biological Sciences, score: 9.48 over 10.
- 07/1995: Master Degree, University of Malaga (Spain). Maximum score.
- 07/1995-01/2001: PhD student on Experimental procedures in Cell and Molecular Biology , University of Malaga (Spain).
- 05/1999-07/1999: research stage at the University of Clermont-1, Clermont-Ferrand, France.
- 05/2000-07/2000: research stage at the University Austral, Valdivia, Chile.
- 01/2001: PhD degree, University of Malaga (Spain). Maximum score.

Awards
- 09/1996: Extraordinary Award for Science Degree (Biology), University of Malaga (Spain).
- 01/1996-12/1999: PhD Fellowship from the Spanish Ministry of Education and Science.

Employment and research experience
- 07/1992-05/2001: undergraduate and graduate student, Laboratory of Animal Physiology, University of Malaga, Spain. Research field: analysis of the secretory products of the subcommissural organ.
- 05/2001-10/2002: Research Associate, Dept. of Anatomy, University of Cambridge, UK. Research field: retinal axon guidance during Xenopus laevis development.
- 10/2002-12/2008: Research Associate, Department of Medicine, University of Cambridge, Cambridge Institute for Medical Research, UK. Research field: cell biology of mutants of neuroserpin and alpha1 antitrypsin that lead to polymerisation and human disease.
- 12/2008-01/2019: permanent researcher (ricercatore) at Department of Biology and Biotechnology Charles Darwin , University of Rome La Sapienza , Italy. Research field: cell biology of mutants of neuroserpin and alpha1 antitrypsin that lead to polymerisation and human disease.
- 01/2019-present: Associate Professor at Department of Biology and Biotechnology Charles Darwin , University of Rome La Sapienza , Italy. Research field: cell biology of mutants of neuroserpin and alpha1 antitrypsin that lead to polymerisation and human disease.

Visiting academic
- 2010, 2011, 2013-17: visiting scientist during the period July-August of each year, University of Cambridge (UK)
- 2018-19: visiting scientist during the period July-August of each year, University College London (UK)

Discipline

Compared anatomy and cytology (BIO/06)

Teaching experience
- 1995-present: teaching of practical sessions to undergraduate students.
- 2002-present: supervision of PhD and undergraduate students.
- 2011-present: responsible for the course Cell Biology (degree on Agroindustrial biotechnologies).
- 2018-present: lecturer for the course Cellular animal biotechnologies and culture systems (degree on Biological sciences).

PhD supervision
- Noemi Guadagno, 11/2012 02/2016 (XXVIII cycle)
- Emanuela D Acunto, 11/2016 02/2020 (XXXII cycle)
- Ilaria Serangeli, 11/2020 (ongoing till 05.2024, XXXVI cycle)

Participation at international meetings
- 09/2019: The 9th International Symposium on Serpin Structure, Function and Biology, Sevilla, Spain. Invited speaker.
- 03/2017: The 8th International Symposium on Serpin Structure, Function and Biology, Shanghai, China. Invited speaker.
- 03-04/2014: The 7th International Symposium on Serpin Structure, Function and Biology, Leogang, Austria. Oral presentation.
- 05/2013: EMBO meeting: The biology of Molecular Chaperones: from Molecules, Organelles and Cells to Misfolding Diseases, Santa Margherita di Pula, Italy. Two posters (last author and coauthor).
- 10/2012: Investigators meeting of the Alpha1 Foundation, Miami, USA. Oral presentation.
- 07/2008: The 5th International Symposium on Serpin Structure, Function and Biology, Leuven, Belgium. Poster (first author).
- 06/2005: The 4th International Symposium on Serpin Structure, Function and Biology, Cairns, Australia. Poster (first author).
- 06/2004: FASEB Conference on Protein misfolding, amyloid and conformational disease . Snowmass, Colorado, USA. Poster (first author).
- 12/2003: 43rd Annual Meeting of The American Society for Cell Biology , San Francisco, USA. Poster (first author).

Membership in scientific societies
- Associazione Biologia Cellulare e del Differenziamento (ABCD), Italy: 2011-2014
- Gruppo Embriologico Italiano (GEI), Italy: 2017-2022

Technical experience
- Cell biology: histological techniques for optic microscopy. Immunocytochemistry with enzymatic and fluorescent probes. Optic and confocal microscopy.
- Cell cultures: culture of adherent and non-adherent cell lines and primary cell cultures. Transient and stable cell transfection.
- Antibody techniques: production of polyclonal and monoclonal antibodies. Purification and isotyping of antibodies. Immunostaining. ELISA. Western Blot. Immunoprecipitation.
- Protein techniques: SDS and native PAGE. Electrotransference and western blot. Protein purification and immunoaffinity purification of proteins. Radioactive pulse-chase with 35S.
- Molecular Biology: DNA purification, molecular cloning, restriction analysis. Generation of constructs for overexpression of genes in eukaryotic cells. PCR.
- Bioinformatics: sequence alignment. PCR primer design.
- Xenopus laevis embryology: production, culture and staging of Xenopus embryos. Microdissections of early embryos. Expose brain preparations. Horseradish peroxidase labeling of Xenopus optic tract.

Languages
Spanish: Native speaker
English: Fluent
Italian: Fluent

Career breaks
- First child born in June 2006, maternity leave from June to October 2006
- Second child born in January 2012, maternity leave from January to May 2012

Grants

- Grant from Sapienza University of Rome. An inducible neuronal model to uncover new toxicity pathways in the neurodegenerative dementia FENIB. Year 2021. Grant amount: euro 14.800. Position: principal investigator.
- Grant from Sapienza University of Rome. Analysis of cytoskeletal alterations due to mutant neuroserpin in a neuronal model of the neurodegenerative dementia FENIB. Year 2020. Grant amount: euro 12.000. Position: principal investigator.
- Grant from Sapienza University of Rome. Mechanisms of cellular toxicity activated by neuroserpin polymers in a neuronal model of the neurodegenerative dementia FENIB. Year 2019. Grant amount: euro 14.500. Position: principal investigator.
- Grant from Sapienza University of Rome. Mitochondrial dysfunction and interaction with the endoplasmic reticulum in a neuronal model of the dementia FENIB. Year 2018. Grant amount: euro 12.000. Position: principal investigator.
- Italian Pasteur Institute-Cenci Bolognetti Foundation. Cellular pathways involved in the toxicity of neuroserpin polymers that cause dementia FENIB. Period: 01/2018-12/2019. Grant amount: euro 40.000. Grant number: 2018-U-14. Position: principal investigator.
- Grant from Sapienza University of Rome. Characterisation of oxidative stress and mitochondrial dysfunction due to neuroserpin polymers in the dementia FENIB. Year 2017. Grant amount: euro 11.000. Position: principal investigator.
- Pilot grant from the Alpha-1 Foundation (USA). Dissecting immunomodulatory mechanisms of A1AT with function-neutralising monoclonal antibodies. Year 2017. Grant amount: $40.000. Position: principal investigator (of a network of three groups, in the UK, Spain and Italy).
- Individual funding for basic research activities (FFABR), Italian Ministry for Education, University and Research (MIUR). Year 2017. Grant amount: euro 3.000.
- Grant from Sapienza University of Rome. Cellular pathways activated by pathological accumulation of neuroserpin polymers in the dementia FENIB. Year 2016. Grant amount: euro 15.000. Position: principal investigator.
- Grant from Sapienza University of Rome. Uncovering new toxicity pathways in the neurodegenerative dementia FENIB. Year 2015. Grant amount: euro 8.400. Position: principal investigator.
- Grant from Sapienza University of Rome. Comparative analysis of the transcriptome in cells expressing wild type or mutant neuroserpin causing dementia FENIB. Year 2014. Grant amount: euro 5.000. Position: principal investigator.
- Grant from Sapienza University of Rome. Characterisation of neurosrepin polymer toxicity in a new neuronal model of the dementia FENIB. Year 2013. Grant amount: euro 7.000. Position: principal investigator.
- Grant from the Telethon Foundation. The role of neuroserpin in familial encephalopathy with neuroserpin inclusion bodies (FENIB). Period: 11/2011 to 11/2014. Grant amount: euro 140.400. Position: co-principal investigator.
- Grant from the Pasteur Institute - Cenci Bolognetti Foundation, Rome, for researchers under 40 years of age. Handling and toxicity of polymerogenic mutants of the serpins within the endoplasmic reticulum. Period: 2011-2013. Grant amount: euro 90.000. Position: principal investigator.
- Grant from Sapienza University of Rome. Mechanisms of cell toxicity of neuroserpin polymers that cause the dementia FENIB. Year 2010. Grant amount: euro 10.000. Position: principal investigator.
- Pilot and feasibility grant from the Alpha1 Foundation. Development of monoclonal antibodies to block the polymerisation of Z a1 antitrypsin. Period: 1/7/2010 to 30/6/2011. Grant amount: $40.000. Position: co-principal investigator.
- Grant from Sapienza University of Rome. Ruolo del Reticolo Endoplasmatico in alterazioni funzionali di proteine neuronali implicate in patologie di tipo neurologico. Year 2009. Grant amount: euro 13.000. Position: principal investigator.
- MRC Programme Grant to D.A. Lomas. Pathobiology of the serpinopathies. Period: 2006-2010 (participated until 2008). Position: research associate.
- MRC Programme Grant to D.A. Lomas. Strategies to prevent cirrhosis and dementia by blocking serpin polymerisation in vivo. Period: 2000-2005. Position: research associate.

Publication metrics (source: Scopus, June 2022)
ORCID ID 0000 0002 0586 8795
- ERC sector: LS3 Cellular and Developmental Biology
- H-index: 27
- Total publication number: 72
- Total citations: 3.558
- Total IF: 556,894
- Number and IF of articles as first author: 8; 57,073
- Number and IF of articles as last or corresponding author: 9; 62,577

URL website
https://corsidilaurea.uniroma1.it/it/users/mariaelenamirandabanosuniroma1it

Publications

Articles

D Acunto E, Gianfrancesco L, Serangeli I, D Orsi M, Sabato V, Guadagno NA, Bhosale G, Caristi S, Failla AV, De Jaco A, Cacci E, Duchen MR, Lupo G, Galliciotti G, Miranda E (2022). Polymeric neuroserpin causes mitochondrial alterations and activates NFkB but not the UPR in a neuronal model of neurodegeneration FENIB. Cellular and Molecular Life Sciences 79:437

Miranda E and Galliciotti G (2022). Editorial: Elucidating the pathological mechanisms of neurodegeneration in the lethal serpinopathy FENIB. Neural Regeneration Research 17:1733

Vapore V, Mazzaglia C, Sibilia D, Del Vecchio M, Fruhmann G, Valenti M, Miranda E, Rinaldi T, Winderickx J, Mazzoni C (2021). Neuroserpin inclusion bodies in a FENIB yeast model. Microorganims 9:1498

Nuñez A, Belmonte I, Miranda E, Barrecheguren M, Farago G, Loeb E, Pons M, Rodriguez-Frias F, Gabriel-Medina P, Rodriguez E, Genescà J, Miravitlles M, Esquinas C (2021). Association between circulating alpha-1 antitrypsin polymers and lung and liver disease. Respiratory Research 22:244

D Acunto E, Fra A, Visentin C, Manno M, Ricagno S, Galliciotti G, Miranda E (2021). Neuroserpin: structure, function, physiology and pathology. Cellular and Molecular Life Sciences 78:6409

Ronzoni R, Ferrarotti I, D Acunto E, Balderacchi AM, Ottaviani S, Lomas DA, Irving JA, Miranda E, Fra AM (2021). The importance of N186 in the alpha-1-antitrypsin shutter region is revealed by the novel Bologna deficiency variant. International Journal of Molecular Sciences 22:5668

Ingwersen T, Linnenberg C, D Acunto E, Temori S, Paolucci I, Wasilewski D, Mohammadi B, Kirchmair J, Glen RC, Miranda E, Glatzel M, Galliciotti G (2021). G392E neuroserpin causing dementia FENIB is secreted from cells but is not synaptotoxic. Scientific Reports 11:8766

Higgins NR, Greenslade JE, Wu JJ, Miranda E, Galliciotti G, Monteiro MJ (2021). Serpin neuropathology in the P497S UBQNL2 mouse model of ALS/FTD. Brain Pathology 29:e12948

Ronzoni R, Heyer-Chauhan N, Fra A, Pearce AC, Rüdiger M, Miranda E, Irving JA, Lomas DA (2021). The molecular species responsible for alpha-1 antitrypsin deficiency are suppressed by a small molecular chaperone. The FEBS Journal, 288:2222-2237

Faull SV, Elliston ELK, Gooptu B, Jagger AM, Aldobiyan I, Redzej A, Badaoui M, Heyer-Chauhan N, Rashid ST, Reynolds GM, Adams DH, Miranda E, Orlova EV, Irving JA, Lomas DA (2020). The structural basis for Z alpha-1 antitrypsin polymerization in the liver. Science Advances, 6:eabc1370

Laffranchi M, Elliston ELK, Miranda E, Perez J, Ronzoni R, Jagger AM, Heyer-Chauhan N, Brantly ML, Fra A, Lomas DA, Irving JA (2020). Intrahepatic heteropolymerisation of M and Z alpha-1-antitrypsin. JCI Insight, 5:e135459

Visentin C, Musso L, Broggini L, Bonato F, Russo R, Moriconi C, Bolognesi M, Miranda E, Dallavalle S, Passarella D, Ricagno S (2020). Embelin as a lead compound for new neuroserpin polymerization inhibitors. Life, 10:111

Visentin C, Broggini L, Sala BM, Russo R, Barbiroli A, Santambrogio C, Nonnis S, Dubnovistky A, Bolognesi M, Miranda E, Achour A, Ricagno S (2020). Glycosylation tunes neuroserpin physiological and pathological properties. International Journal of Molecular Sciences, 21:E3235

Mela M, Smeeton W, Davies SE, Miranda E, Scarpini C, Coleman N, Alexander GJM (2020). The alpha-1 antitrypsin polymer load correlates with hepatocyte senescence, fibrosis stage and liver-related mortality. Chronic Obstructive Pulmonary Diseases, 7:151-162

Trobiani L, Favaloro FL, Di Castro MA, Di Mattia M, Cariello M, Miranda E, Canterini S, De Stefano ME, Comoletti D, Limatola C, De Jaco A (2018). UPR activation specifically modulates glutamate neurotransmission in the cerebellum of a mouse model of autism. Neurobiology of Disease, 120:139-150

Fra A, D Acunto E, Laffranchi M, Miranda E (2018). Cellular models for the serpinopathies. In: Serpins: methods and protocols. Methods in Molecular Biology, 1826:109-121

Bazzan E, Tinè M, Biondini D, Benetti R, Baraldo S, Turato G, Fagiuoli S, Sonzogni A, Rigobello C, Rea F, Calabrese F, Foschino-Barbaro MP, Miranda E, Lomas DA, Saetta M, Cosio MG (2018). Alpha1-antitrypsin polymerizes in alveolar macrophages of smokers with and without alpha1-antitrypsin deficiency. Chest, 154(3):607-616

Miranda E, Ferrarotti I, Berardelli R, Laffranchi M, Cerea M, Gangemi F, Haq I, Ottaviani S, Lomas DA, Irving JA, Fra A (2017). The pathological Trento variant of alpha-1 antitrypsin (E75V) shows non-classical behaviour during polymerisation. FEBS Journal, 284(13):2110-2126

Guadagno NA, Miranda E (2017). Editorial: Polymer toxicity in neurodegeneration FENIB. Oncotarget, 8(22):35490-35491

Guadagno NA, Moriconi C, Licursi V, D Acunto E, Nisi P, Carucci N, De Jaco A, Cacci E, Negri R, Lupo G, Miranda E (2017). Neuroserpin polymers cause oxidative stress in a neuronal model of the dementia FENIB. Neurobiology of Disease, 103:32-44

Motamedi-Shad N, Jagger AM, Liedtke M, Faull SV, Nanda AS, Salvadori E, Wort JL, Kay CW, Heyer-Chauhan N, Miranda E, Perez J, Ordóñez A, Haq I, Irving JA, Lomas DA (2016). An antibody that prevents serpin polymerisation acts by inducing a novel allosteric behaviour. Biochem Journal, 473(19):3269-3290

Fra A, Cosmi F, Ordoñez A, Berardelli R, Perez J, Guadagno NA, Corda L, Marciniak SJ, Lomas DA, Miranda E (2016). Polymers of Z alpha-1 antitrypsin are secreted in cell models of disease. European Respiratory Journal, 47(3):1005-1009

Saga G, Sessa F, Barbiroli A, Santambrogio C, Russo R, Sala M, Raccosta S, Martorana V, Caccia S, Noto R, Moriconi C, Miranda E, Grandori R, Manno M, Bolognesi M, Ricagno S (2016). Embelin binds to human neuroserpin and impairs its pathologic polymerisation. Scientific Reports, 6:18769

Moriconi C, Ordoñez A, Lupo G, Gooptu B, Irving JA, Noto R, Martorana V, Manno M, Timpano V, Guadagno NA, Dalton L, Marciniak S, Lomas DA, Miranda E (2015). Interactions between N-linked glycosylation and polymerisation of neuroserpin within the endoplasmic reticulum. FEBS Journal, 282(23):4565-4579

Ordóñez A, Pérez J, Tan L, Dickens JA, Motamedi-Shad N, Irving JA, Haq I, Ekeowa U, Marciniak SJ, Miranda E§, Lomas DA§ (2015). A single-chain variable fragment intrabody prevents intracellular polymerisation of Z alpha1-antitrypsin while allowing its antiproteinase activity. The FASEB Journal, 20(6):2667-2678
§Joint senior authors.

Irving JA, Miranda E, Haq I, Perez J, Kotov VR, Faull SV, Motamedi-Shad N, Lomas DA (2015). An antibody raised against a pathogenic serpin variant induces mutant-like behaviour in the wild-type protein. The Biochemical Journal, 468(1):99-108

Noto R, Randazzo L, Raccosta S, Caccia S, Moriconi C, Miranda E, Martorana V, Manno M (2015). The stability and activity of human neuroserpin are modulated by a salt bridge that stabilises the reactive centre loop. Scientific Reports, 5:13666

Preissler S, Chambers JE, Crespillo-Casado A, Avezov E, Miranda E, Perez J, Hendershot LM, Harding HP, Ron D (2015). Physiological modulation of BiP activity by trans-promoter engagement of the interdomain linker. eLife, 4:e08961

Tan L, Perez J, Mela M, Miranda E, Burling KA, Rouhani FN, DeMeo DL, Haq I, Irving JA, Ordoñez A, Dickens JA, Brantly M, Marciniak SJ, Alexander GJM, Gooptu B, Lomas DA (2015). Characterising the association of latency with alpha1-antitrypsin polymerisation using a novel monoclonal antibody. International Journal of Biochemistry and Cell Biology, 58:81-91

Tan L, Dickens JA, Demeo DL, Miranda E, Perez J, Rashid ST, Day J, Ordoñez A, Marciniak SJ, Haq I, Barker AF, Campbell EJ, Eden E, McElvaney NG, Rennard SI, Sandhaus RA, Stocks JM, Stoller JK, Strange C, Turino G, Rouhani FN, Brantly M, Lomas DA (2014). Circulating polymers in alpha1-antitrypsin deficiency. European Respiratory Journal, 43(5):1501-1404

Ljujic M, Rankov AD, Kojic S, Miranda E, Radojkovic D (2014). Functional analysis of novel alpha-1 antitrypsin variants G320R and V321F. Molecular Biology Reports, 41(9):6133-6141

Roussel BD, Kruppa AJ, Miranda E, Crowther DC, Lomas DA, Marciniak SJ (2013). Endoplasmic reticulum dysfunction in neurological disease. The Lancet Neurology, 12(1):105-118

Ordoñez A, Snapp EL, Tan L, Miranda E, Marciniak SJ, Lomas DA (2013). Endoplasmic reticulum polymers impair luminal protein mobility and sensitise to cellular stress in alpha(1)-deficiency. Hepatology, 57(5):2049-2060

Schipanski A, Lange S, Segref A, Gutschmidt A, Lomas DA, Miranda E, Schweizer M, Hoppe T, Glatzel M (2013). A novel interaction between aging and ER overload in a protein conformational dementia. Genetics, 193(3):865-876

Lupo G, Novorol C, Smith JR, Vallier L, Miranda E, Alexander M, Biagioni S, Pedersen RA, Harris WA (2013). Multiple roles of activin/nodal, bone morphogenetic protein, fibroblast growth factor and Wnt/beta-catenin signalling in the anterior neural patterning of adherent human embryonic stem cell cultures. Open Biology, 3(4):120167

Fra AM, Gooptu B, Ferrarotti I, Miranda E, Scabini R, Ronzoni R, Benini F, Corda L, Medicina D, Luisetti M, Schiaffonati L (2012). Three new alpha1-antitrypsin deficiency variants help to define a C-terminal region regulating conformational change and polymerization. Plos One, 7(6):e38405

Schmid S, Koepke J, Dresel M, Hattesohl A, Frenzel E, Perez J, Lomas D, Miranda E, Greulich T, Noeske S, Wencker M, Teschler H, Vogelmeier C, Janciauskiene S, Koczulla A (2012). The effects of weekly augmentation therapy in patients with PiZZ 1-antitrypsin deficiency. International Journal Chronic Obstructive Pulmonary Disease, 7:687-696

Yusa K, Rashid ST, Strick-Marchand H, Varela I, Liu PQ, Paschon DE, Miranda E, Ordóñez A, Hannan NR, Rouhani FJ, Darche S, Alexander G, Marciniak SJ, Fusaki N, Hasegawa M, Holmes MC, Di Santo JP, Lomas DA, Bradley A, Vallier L (2011). Targeted gene correction of alpha1-antitrypsin deficiency in induced pluripotent stem cells. Nature, 478(7369):391-394

Morris H, Morgan MD, Wood AM, Smith SW, Ekeowa UI, Herrmann K, Holle JU, Guillevin L, Lomas DA, Perez J, Pusey CD, Salama AD, Stockley R, Wieczorek S, McKnight AJ, Maxwell AP, Miranda E, Williams J, Savage CO, Harper L (2011). ANCA-associated vasculitis is linked to carriage of the Z allele of alpha1-antitrypsin and its polymers. Annals of the Rheumatic Diseases, 70(10):1851-1856

Rodriguez-Gonzalez R, Sobrino T, Rodriguez-Yanez M, Millan M, Brea D, Miranda E, Moldes O, Perez J, Lomas DA, Leira R, Davalos A, Castillo J (2011). Association between neuroserpin and molecular markers of brain damage in patients with acute ischemic stroke. Journal of Translational Medicine, 9(1):58

Rodríguez-González R, Millán M, Sobrino T, Miranda E, Brea D, Perez de la Ossa N, Blanco M, Pérez J, Dorado L, Castellanos M, Lomas DA, Moro MA, Dávalos A, Castillo J (2011). The natural tissue plasminogen activator inhibitor neuroserpin and acute ischemic stroke outcome. Journal of Thrombosis and Haemostasis, 105(3):421-429

Chang YP; Mahadeva R, Patschull AO, Nobeli I, Ekeowa UI, McKay AR, Thalassinos K, Irving JA, Haq I, Nyon MP, Christodoulou J, Ordoñez A, Miranda E, Gooptu B (2011). Targeting serpins in high-throughput and structure-based drug design. Methods in Enzymology, 501:139-175

Irving JA, Ekeowa UI, Belorgey D, Haq I, Gooptu B, Miranda E, Perez J, Roussel BD, Ordoñez A, Dalton LE, Thomas SE, Marciniak SJ, Parfrey H, Chilvers ER, Teckman JH, Alam S, Mahadeva R, Rashid ST, Vallier L, Lomas DA (2011). The serpinopathies: studying serpin polymerization in vivo. Methods in Enzymology, 501: 421-466

Duvoix A, Miranda E, Perez J, Sorensen GL, Holmskov U, Trapnell BC, Madsen J, Clark HW, Edwards LD, Miller BE, Tal-Singer RM, Lomas DA (2011). Evaluation of full-length, cleaved and nitrosylated serum surfactant protein D as biomarkers for COPD. COPD, 8(2):79-95

Belorgey D, Irving JA, Ekeowa UI, Freeke J, Roussel BD, Miranda E, Perez J, Marciniak SJ, Crowther DC, Michel CH, Lomas DA (2011). Characterisation of serpin polymers in vitro and in vivo. Methods, 53(3):255-266

Ekeowa UI*, Freeke J*, Miranda E*, Gooptu B, Bush MF, Pérez J, Teckman J, Robinson CV and Lomas DA. Defining the mechanism of polymerization in the serpinopathies (2010). PNAS, 107(40):17146-17151
*Joint first authors

Miranda E, Perez J, Ekeowa U, Hadzic N, Kalsheker N, Gooptu B, Portmann B, Belorgey D, Hill M, Chambers S, Teckman J, Alexander G, Marciniak S, Lomas D (2010). A novel monoclonal antibody to characterize pathogenic polymers in liver disease associated with alpha1-antitrypsin deficiency. Hepatology, 52(3):1078-1088

Rashid ST, Corbineau S, Hannan N, Marciniak SJ, Miranda E, Alexander G, Huang Doran I, Ahrlund-Richter L, Skepper J, Griffin J, Semple R, Weber A, Lomas DA, Vallier L (2010). Modelling inherited metabolic disorders of the liver with human induced pluripotent stem cells. Journal of Clinical Investigations, 120(9):3127-3136

Kröger H, Miranda E, MacLeod I, Pérez J, Crowther DC, Marciniak SJ and Lomas DA (2009). ERAD and autophagy cooperate to degrade polymerogenic mutant serpins. Journal of Biological Chemistry, 284(34):22793-22802

Davies MJ, Miranda E, Marciniak SJ, Kaufman RJ, Römisch K and Lomas DA (2009). Neuroserpin polymers activate NF- B by a calcium signalling pathway that is independent of the unfolded protein response. Journal of Biological Chemistry: 284(27):18202-18209

Medicina D, Montani N, Tiberio L, Fra AM, Corda L, Miranda E, Pezzini A, Bonetti F, Ferrarotti I, Facchetti F and Schiaffonati L (2009). Molecular characterization of the new defective Pbrescia alpha1-antitrypsin allele. Human Mutation, 30(8):E771-E781

Gooptu B, Miranda E, Nobeli I, Mallya M, Purkiss A, Leigh Brown SC, Summers C, Phillips RL, Lomas DA, Barrett TE (2009). Crystallographic and cellular characterisation of two mutations stabilising the native fold of alpha1-antitrypsin: implications for rational drug design. Journal of Molecular Biology: 387(4):857-868

Ekeowa UI, Gooptu B, Belorgey D, Hägglöf P, Karlsson-Li S, Miranda E, Pérez J, MacLeod I, Kroger H, Marciniak SJ, Crowther DC and Lomas DA (2009). Alpha1-antitrypsin deficiency, chronic obstructive pulmonary disease and the serpinopathies. Clinical Science: 116(12):837-850

Miranda E, MacLeod I, Davis MJ, Pérez J, Römisch K, Crowther D and Lomas DA (2008). The intracellular accumulation of polymeric neuroserpin explains the severity of the dementia FENIB. Human Molecular Genetics: 17(11):1527-1539

Nielsen HM, Minthon L, Londos E, Blennow K, Miranda E, Perez J, Crowther DC, Lomas DA and Janciauskiene SM (2007). Plasma and cerebrospinal fluid serpins in Alzheimer s disease and
dementia with Lewy bodies. Neurology: 69(16):1569-1579

Kennedy S, van Diepen A, van den Hurk C, Coates LC, Lee TW, Ostrovsky LL, Miranda E, Perez J, Davies MJ, Lomas DA, Dunbar PR and Birch NP (2007). Expression of the serine protease inhibitor neuroserpin in cells of the human myeloid lineage. Thrombosis and Haemostasis: 97(3): 394-399

Miranda E and Lomas DA (2006). Neuroserpin: a serpin to think about. Cellular and Molecular Life Sciences :63(6):709-722

Hoyo-Becerra C, Lopez-Avalos MD, Perez J, Miranda E, Rojas-Rios P, Fernandez-Llebrez P, Grondona JM (2006). Continuous delivery of a monoclonal antibody against Reissner s fiber into CSF reveals CSF-soluble material immunorelated to the subcommisssural organ in early chick embryos. Cell and Tissue Research: 326:771-786

Crowther DC, Kinghorn KJ, Miranda E, Page R, Curry JA, Duthie F, Gubb D, Lomas DA (2005). Intraneuronal A-beta, non-amyloid aggregates and neurodegeneration in a Drosophila model of Alzheimer s disease. Neuroscience: 132:123-135

Lomas DA, Belorgey D, Mallya M, Miranda E, Kinghorn KJ, Sharp LK, Phillips RL, Page R, Robertson AS, Crowther DC (2005). Molecular mousetraps and the serpinopathies. GlaxoSmithKline Award Lecture published in Biochemical Society Transactions: 33(2):321-330

Miranda E, Romisch K, Lomas DA (2004). Mutants of neuroserpin that cause dementia accumulate as polymers within the endoplasmic reticulum. Journal of Biological Chemistry: 279(27):28283-28291

Lomas DA, Belorgey D, Mallya M, Onda M, Kinghorn KJ, Sharp LK, Phillips RL, Page R, Crowther DC, Miranda E (2004). Polymerisation underlies alpha1-antitrypsin deficiency, dementia and other serpinopathies. Review. Frontiers in Bioscience: 9:2873-2891

Crowther DC, Belorgey D, Miranda E, Kinghorn KJ, Sharp LK, Lomas DA (2004). Practical genetics: alpha-1-antitrypsin deficiency and the serpinopathies. Review. European Journal of Human Genetics: 12(3):167-172

Mann F*, Miranda E*, Weinl C*, Harmer E, Holt CE (2003). B-type Eph receptors and ephrins induce growth cone collapse through distinct intracellular pathways. Journal of Neurobiology: 57(3):323-336
*Joint first authors

Miranda E, Almonacid JA, Rodriguez S, Perez J, Hein S, Cifuentes M, Fernandez-Llebrez P, Rodriguez EM (2001). Searching for specific binding sites of the secretory glycoproteins of the subcommissural organ. Microscopy Research and Technique: 1;52(5):541-551

Fernandez-Llebrez P, Miranda E, Estivill-Torrus G, Cifuentes M, Grondona JM, Lopez-Avalos MD, Perez-Martin M, Perez J (2001). Analysis and quantification of the secretory products of the subcommissural organ by use of monoclonal antibodies. Microscopic Research Techniques: 1;52(5):510-519

Arrabal PM, Estivill-Torrus G, Miranda E, Perez J, Fernandez-Llebrez P (2000). Identification of Reissner's fiber-like glycoproteins in two species of freshwater planarians (Tricladida), by use of specific polyclonal and monoclonal antibodies. Cell and Tissue Reseach: 300(3):427-434

Estivill-Torrus G, Cifuentes M, Grondona JM, Miranda E, Bermudez-Silva FJ, Fernandez-Llebrez P, Perez J (1998). Quantification of the secretory glycoproteins of the subcommissural organ by a sensitive sandwich ELISA with a polyclonal antibody and a set of monoclonal antibodies against the bovine Reissner's fiber. Cell and Tissue Research: 294(3):407-413

Lopez-Avalos MD, Cifuentes M, Grondona JM, Miranda E, Perez J, Fernandez-Llebrez P (1997). Rostral floor plate (flexural organ) secretes glycoproteins immunologically similar to subcommissural organ glycoproteins in dogfish (Scyliorhinus canicula) embryos. Brain Research and Developmental Brain Research: 18;102(1):69-75

Book chapters

Galliciotti G, De Jaco A, Sepulveda-Falla D, D Acunto E, Miranda E (2020). Role of cellular oxidative stress in dementia. Book chapter in: The neuroscience of dementia. Editors: Colin Martin and Victor Preedy. Elsevier (San Diego, USA)

Lomas DA, Belorgey D, Miranda E, Mallya M, Hagglof P, Sharp LK, Phillips RL, Page R, Davies MJ, Crowther DC (2007). The serpinopathies and respiratory disease. In Molecular and Cellular Aspects of the serpinopathies and disorders in serpin activity. Eds. Silverman GA and Lomas DA. World Scientific, pp. 445-482

Lomas DA, Belorgey D, Mallya M, Onda M, Kinghorn KJ, Sharp LK, Phillips RL, Page R, Miranda E, Crowther DC (2004). Serpinopathies and the central nervous system. In Amyloid and Amyloidosis. Eds. G. Grateau, R. A. Kyle and M. Skinner. London, CRC Press, pp. 388-390