EUROSERPIN2023 Programme

Grand Hotel Piazza Borsa, Palermo 5-7 June 2023

Monday 5^th June 2023

8:30-9:00        Registration

9:00-9:30        Welcome address - James Irving (University College London, UK)

Session 1 - Serpins in health and disease I

9:30-9:45        Robin Carrell (University of Cambridge, UK)

Serpins: hormones, tree frogs and fevers

9:45-10:15      Sabina Janciauskiene (Hannover Medical School, Germany)

Origin and biological significance of molecular forms of alpha-1-antitrypsin

10:15-10:45    Giovanna Galliciotti (UKE Hamburg, Germany)

The double life of neuroserpin: investigating inhibitory and non-inhibitory mechanisms

10:45-11:15    Coffee break

11:15-11:45    Julia Peñas Martinez (University of Murcia, Spain)

Anti-tumor role of antithrombin in glioblastoma

11:45-12:15    Riccardo Ronzoni (University College London, UK)

Evaluation of polymer fate in ex vivo patient-derived organoids expressing Z alpha-1 antitrypsin

12:15-12:30    John T. Heiker (University of Leipzig and University Hospital Leipzig, Germany)

Vaspin and the regulation of adipose tissue metabolism – links to energy expenditure

12:30-12:45    Santina Quarta (University of Padova, Italy)

Inhibition of proteinase activated receptor-2 activation in neurodegenerative diseases

12:45-14:00    Free time for lunch

Session 2 - Therapies and Disease models

14:00-14:45    David Lomas (University College London, UK)

A small molecule therapy for AAT deficiency

14:45-15:15    Tamir Rashid (Imperial College London, UK)

Human iPSCs for therapeutic target discovery in PiZ Alpha1 Antitrypsin deficiency

15:15-15:45    Nicola Elvassore (University of Padova, Italy)

Modelling Alpha1-Antitrypsin deficiency using human liver organoids

15:45-16:15    Coffee break

16:15-16:45    Joseph Chambers (University of Cambridge, UK)

Polymers as molecular filters: endoplasmic reticulum biophysics in alpha-1 antitrypsin deficiency

16:45-17:15    Patrizia Pontisso (University of Padova, Italy)

The CCAAT/enhancer-binding protein beta - SerpinB3 axis inhibition as a novel strategy for non-alcoholic steatohepatitis treatment

17:15-17:30    Tiziana Cremona (University of Bern, Switzerland)

Cell specific gene editing for treatment of alpha-1 antitrypsin deficiency

17:30              Group picture and guided tour of the historical centre of Palermo

Tuesday 6^th June 2023

Session 3 - Serpins in health and disease II

9:00-9:30        Pasquale Piccolo (TIGEM, Naples, Italy)

Mechanisms of liver disease due to Z alpha-1 antitrypsin

9:30-10:00      Ilaria Ferrarotti (University of Pavia, Italy)

Clinical impact of rare alpha-1 antitrypsin variants

10:00-10:15    Valentina Barzon (University of Pavia, Italy)

Testing the novel Q0genova deletion in patient with alpha-1 antitrypsin deficiency

10:15-10:30    Andrea Denardo  (University of Brescia, Italy)

Mutations in the reactive centre loop and alpha-1 antitrypsin inhibitory activity

10:30-11:00    Coffee break

11:00-11:30    Alexandra Lucas (Arizona State University, USA)

Treatment with anti-inflammatory viral serpin modulates immuno-thrombotic responses in mouse models of severe disease

11:30-12:00    Marie-Christine Bouton (INSERM Paris, France)

Protease nexin-1, a unique serpin in hemostasis, thrombosis and inflammation

12:00-12:15    Ilaria Serangeli (Sapienza University of Rome, Italy)

Mutant neuroserpin induces mitochondrial and cytoskeleton alterations in a neuronal model of neurodegeneration FENIB

12:15 -12:30   Kamila Kamuda (University College London, UK)

Changes in the cellular landscape of elicited by expression of Z variant of alpha-1 antitrypsin

12:30-14:00    Free time for lunch

Session 4 - Structural and molecular mechanisms

14:00-14:30    Lisa Cabrita (University College London, UK)

Characterisation studies of the early biosynthesis and co-translational folding of alpha-1 antitrypsin

14:30-14:45    Giuseppe Legname (SISSA, Trieste, Italy)

SERPINA3/SerpinA3n involvement in prion diseases

14:45-15:00    Sarah Vickers (University College London, UK)

Investigating misfolding of alpha-1 antitrypsin using ion mobility mass spectrometry

15:00-15:15    Samuele Raccosta (CNR, Palermo, Italy)

Scaling concepts in serpin polymer

15:15-15:45    Coffee break

15:45-16:15    Adriana Ordoñez (University of Cambridge, UK)

A CRISPR-Cas9 whole genome screen identifies new regulators in the secretion of polymerogenic alpha-1 antitrypsin

16:15-16:30    Cristina Visentin (University of Milan, Italy)

Neuroserpin: strategies to prevent polymerisation and new expression protocol         

16:30-16:45    Ibrahim Aldobiyan (University College London, UK)

The molecular basis of pathological alpha-1 antitrypsin polymerisation

16:45-17:00    Sarah Lowen (University College London, UK)

Characterisation of the alpha-1 antitrypsin pathway in atomic detail by NMR spectroscopy

17:00-17:15    Charlie Hitchman (University of Leicester, UK)

Structural studies of endoplasmic reticulum enzymes that interact with misfolding and polymeric Z alpha-1 antitrypsin

17:30-19:00    Poster session

20:00              Meeting dinner

Wednesday 7^th June 2023

Session 5 - Serpins in health and disease III

Please note the later start

9:30-10:00        Nunzia Pastore (TIGEM, Naples, Italy)

Increased expression or activation of TRPML1 reduces hepatic storage of toxic Z alpha-1 antitrypsin

10:00-10:30      Benoit Roussel (INSERM Caen, France)

Tissue-type plasminogen activator: to infinity and beyond!

10:30-11:00    Coffee break

Session 6 - Applications, tools and new perspectives

11:00-11:30    James Irving (University College London, UK)

Ligand binding reveals molecular changes that occur during the polymerisation of alpha-1 antitrypsin

11:30-11:45    Annamaria Fra (University of Brescia, Italy)

Circulating polymers and anti-elastase activity in AATD

11:45-12:00    Annalisa Radeghieri (University of Brescia, Italy)

Extracellular nanoparticles, biomolecular corona and antithrombin         

12:00-12:15    Monica Chinellato (University of Padova, Italy)

Inhibiting the inhibitor: development of nanobodies to block SERPINB3 in dysregulated hepatic context

12:15-12:30    Elena Miranda (Sapienza University of Rome, Italy)

Analysis of novel variants of neuroserpin and application of pathogenicity predictors for the discovery of new mutations that cause neurodegeneration FENIB

12:30-13:00    Concluding remarks

Current as at May 19th 2023