Centre de Référence Maladies Rares

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Biotherapy of genetic diseases, inflammatory disorders and cancers (BMGIC)

FR: Biologie Fondamentale Appliquée à la Médecine

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2019

ESDR meeting 2019

The European Society for Dermatological Research (ESDR) meeting will take place in Bordeaux in 2019 from 18 to 21 september

2018

FR TRANSBIOMED CONFERENCE : Bill Martin invited by Zoran Ivanovic

Bill Martin conference

11h amphithéâtre Broca Neurosciences, Carreire zone sud
Bill Martin
Institute of Molecular Evolution – Heinrich-Heine-Universitaet Duesseldorf – Germany

“Anaerobisis and evolution: We should not forget our humble anaerobic beginnings”
Today the atmosphere has about 21% oxygen. Earth scientists now tell us, however, that the rise of oxygen was a very late event in evolution, something that occurred in the last 600 million or even the last 450 million years, since the origin of land plants. Life on land is dominated by eukaryotes, land plants and of course land animals. Before life on land, life was confined to aquatic environments. But eukaryotes have a fossil record that extends back more than 1.5 billion years. The majority of eukaryote evolution took place in anaerobic environments. Thus it is should not be surprising to see that the traces of our anaerobic past preserved in diverse eukaryotic lineages. Part of the talk will be devoted to looking at the remnants of our anaerobic past as preserved in modern metabolis, both in anaerobes and in aerobes. A puzzling aspect of oxygen in Earth history is this: Cyanobacteria started making molecular oxygen about 2.5 billion years ago, but why did it take so long for oxygen to accumulate in the atmosphere? There are a number of very complicated proposals in the literature. Perhaps the reason is very simple, though, and we will consider such a suggestion (nitrogenase inhibition). Perhaps the reason is simple enough that everyone (nonspecialists) can discuss it.

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FR TransbioMed cobference : Franck Antonicelli invited by Katia Boniface

14h, salle de conférence IBGC (Carreire zone sud)
Frank Antonicelli
EA-7319 Derm-I-C , Laboratoire de Dermatologie , Reims

Sujet : “La pemphigoïde bulleuse : du lit au lit en passant par la paillasse”

La pemphigoïde bulleuse (PB) est la plus fréquente des dermatoses bulleuses auto-immunes. Elle touche avec prédilection le sujet âgé et se caractérise cliniquement par des bulles cutanées sur base inflammatoire. Son pronostic est médiocre (mortalité>20% à 12 mois), malgré les progrès thérapeutiques basés sur une dermocorticothérapie forte (DCF), qui est désormais le traitement de référence de la PB quelle que soit sa sévérité. De même, le nombre de patients qui rechutent durant la 1ere année de suivi reste élevé (environ 30%). Des progrès majeurs ont été réalisés ces dernières décennies sur le diagnostic immuno-pathologique et la compréhension des mécanismes physiopathologiques liés à la PB. Le rôle pathogène des anticorps anti-PB180 est désormais bien établi chez l’homme. Des modèles murins ont permis de reproduire la maladie et d’élucider le rôle pathologique des auto-anticorps comme inducteurs de la réaction inflammatoire qui est à l’origine de la formation de la bulle. Toutefois la transposition des résultats murins vers l’homme présente de nombreuses limites et ne permet pas d’expliquer pourquoi certains patients développent cette maladie et d’autres pas. De même, les questions restent sur les facteurs influençant la rechute, ainsi que ceux influençant la variabilité dans la présentation clinique au moment du diagnostic. Depuis plusieurs années, nos travaux se concentrent sur l’étude de ces variabilités interindividuelles, et l’utilisation de modèles ex vivo a permis d’améliorer non seulement nos connaissances sur les mécanismes physiopathologiques de cette maladie au moment du diagnostic mais aussi sur les facteurs de risque de rechute, le but ultime étant toujours d’apporter de nouvelles options thérapeutiques pour ces patients fragiles.

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Annual Conference FR TransBioMed

The Annual Conference of the FR TransBioMed will take place at the Agora, Domaine du Haut Carré (Pessac)
Registration at this link

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FR Transbiomed conference : Hervé Seitz, invited by François Moreau-Gaudry

The 26 November 2018 from 11:00 to 12:00.

Hervé Seitz, Montpellier (IGH). Impact systémique des petits ARN régulateurs

Gene regulation is traditionally assessed by molecular assays, probing the physical interaction between regulators and their targets (transcription factors with promoters, RNA-binding proteins with mRNAs, …). The advent of high-throughput techniques now allows a sensitive detection of molecular interactors for the regulators of interest – and these experiments usually identify hundreds or thousands of targets per regulator. This is particularly true for microRNAs (“miRNAs”), which are therefore believed to control most coding genes in mammals. Functional importance of these interactions is apparently confirmed by comparative genomics, which suggests that many miRNA binding sites are conserved in evolution. Yet several observations suggest that miRNAs do not have such a broad biological effect; in particular, miRNA-guided repression is surprisingly small (targets are usually repressed less than 2-fold, and gene activity is typically insensitive to such small changes in gene expression). We thus decided to revisit the biological relevance of miRNA/target interactions, and our results are indeed inconsistent with the notion that miRNAs regulate functionally so many genes. It appears that molecular biology techniques, as well as computational predictions, tend to over-estimate broadly the miRNA target pool, and just a subset of the identified targets are really, functionally targeted. Other regulatory events appear to be buffered by homeostasis, and they are inconsequential at the macroscopic scale. These notions are generalizable to every type of gene regulator, and they could promote a profound redefinition of “gene regulation”.

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2nd symposium of inflammatory and cancer in Dermatology in Nouvelle-Aquitaine

in the University of Poitiers.

registration at this link

SCIENTIFIC MEETING Fondation René Touraine 2018

SCIENTIFIC MEETING 2018:
THE ROLE OF THE EXTRACELLULAR MATRIX IN SKIN PHYSIOLOGY AND PATHOLOGY

Friday 16th November 2018, in Paris,
at Musée des Moulages de l’Hôpital Saint-Louis,
1 avenue Claude Vellefaux – 75010 Paris.

Invited speackers :
Ryan R DRISKELL (Pullman, US)
Rachel WATSON (Manchester, United Kingdom)
Gary FISHER (Ann Arbor, US)
Jose Luis JORCANO (Madrid, Spain)
Gian-Paolo DOTTO (Lausanne, Switzerland)
Beate ECKES (Cologne, Germany)
Teresa ODORISIO (Rome, Italy)
Jagdeep NANCHAHAL (Oxford, United Kingdom)

Inscriptions:
Fondation René Touraine, Hôpital St Louis, Pavillon Bazin,
1, avenue Claude Vellefaux – F-75475- PARIS cedex 10 – (France)
Téléphone : 01 53 72 20 60 Fax : 01 53 72 20 61 courriel: christina.pitton@fondation-r-touraine.org
Or in the website : http://www.fondation-r-touraine.org/Journees-scientifiques#sommaire

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A trophy for Aksam Merched

Aksam Merched Trophy

The 20th of octobre in Tripoli (Liban), Pr. Aksam Merched, from MiRCaDe team, receive a trophy during the 24th Congress of Surgical Society of North Leabanon, from the organizing committee and his president for accepting their invitation and giving a lecture on immunotherapy of cancer in a special session dedicated to therapeutic innovations.

Alain Taïeb will be awarded the first new ESPCR Award at the EPSCR meeting

Alain Taïeb will be awarded the first new ESPCR Award at the EPSCR meeting on 24 september in Rennes. ESPCRblog

The European Society for Pigment Cell Research (ESPCR) is a scientific society dedicated to understanding the biology of the pigment cells, melanocytes, from normal to pathological development.

The 21th ESPCR Meeting will be hosted in Rennes, the capital of Brittany in France on 24 to 27 september 2018.

It aims at deciphering the biology of the melanocyte and its ecosystem, the better to understand its dysfunction and pathological evolution.

To this end, the 21th ESPCR Meeting will bring together competencies from basic science to disease. It will also be a unique opportunity to envision the forthcoming new therapeutic approaches.

To promote science, the ESPCR meeting includes two keynote lectures, guest lectures from internationally recognized scientists, oral presentations from peer-reviewed abstracts, poster sessions and an Innovation pitches session.

Social events are organized to promote networking and very importantly, the ESPCR sponsors several travel awards.

Prof Alain Taïeb, Chairman of the International Society of Atopic Dermatitis (ISAD)

Prof Alain Taïeb is elected Chairman of the International Society of Atopic Dermatitis (ISAD)

Bordeaux Cell Biology Gathering (BCBG)

the second edition of BCBG will take place the 4th of April at the Domaine du Haut-carré (Talence) from 9 a.m to 5 p.m.

The meeting is free but inscription is mandatory
http://www.transbiomed.u-bordeaux.fr/bcbg/bcbg-inscription

More info :
http://www.transbiomed.u-bordeaux.fr/bcbg/infos-pratiques

Conference Pr David Kelsell invité par Alain Taïeb

Conférence FR Transbiomed : Pr David Kelsell invité par Alain Taïeb, jeudi 1er mars 2018 à 11h
iRhom2 and the keratinocyte stress response

David Kelsell, Professor of Human Molecular Genetics, Blizard Institute, Barts and the London Medical School, Queen Mary University of London

Biosketch :
My research largely focuses on the molecular and cellular mechanisms underlying inherited non-syndromic and syndromic skin disease including keratodermas, ichthyoses and atopic eczema. In addition, this work has highlighted the importance of studying skin disease to understand non-cutaneous conditions such as oesophageal cancer, hearing loss and cardiomyopathy. I am Deputy Dean for Research of Barts and the London Medical School, and I am President-Elect of the European Society of Dermatological Research. I was awarded the 2016 CHANEL CERIES research award.

Abstract : The ability of cells and tissues to withstand and respond to a variety of stressors is fundamental to life. In mammals, the skin plays a unique role in the defence against external damage, pathogens and allergens, and therefore displays many specialised adaptations to environmental stress. In particular, the epidermis of palmoplantar (palm and sole) regions is uniquely adapted to withstand remarkable physical stress. We have identified dominantly-inherited point mutations in the gene encoding iRhom2 (RHBDF2) associated with Tylosis with Oesophageal Cancer, a syndrome in which affected individuals develop focal palmoplantar keratoderma (PPK) and have a very high risk of oesophageal squamous cell carcinoma. We have shown these point mutations to result in gain-of-function of the iRhom2 protein, a catalytically inactive member of the rhomboid family. Based on multiple lines of evidence generated by our laboratory, this proposal hypothesises that iRhom2 functions as a major regulator of the response to cellular stress and disease in the epidermis. Our work has shown iRhom2 to be an important regulator of keratin dynamics, the epidermal barrier and growth factor / cytokine production via regulation of the sheddase ADAM17. This talk will discuss the role of iRhom2 in regulating epidermal keratin dynamics in response to stress and will describe signalling pathways both upstream and downstream of iRhom2.

conference Jeremy Di Domizio

Jeremy Di Domizio

conference FR Transbiomed :
Jeremy Di Domizio invited by Katia Boniface
tuesday, 20 february, 14h
conference room at the Plateforme de Génomique Fonctionnelle, Carreire, University of Bordeaux.

Jeremy Di Domizio
University Hospital of Lausanne CHUV, Lausanne, Switzerland

Pathogenic role of interleukin (IL)-26 producing Th17 cells in the acute forms of psoriasis

Chronic plaque-type psoriasis is a skin inflammatory disease driven by IL-17-producing Th17 cells. In addition to IL-17, Th17 cells in psoriasis produce other cytokines including interleukin (IL)-26, a member of IL-10 cytokine family. However, whether the production of IL-26 by Th17 cells plays a pathogenic role in psoriasis is unknown. By comparing IL-26 and IL-17 expression in psoriatic skin samples, we found that high IL-26 and low IL-17 were detected in acute forms of psoriasis, including erythrodermic and guttate-type, whereas low IL-26 and high IL-17 mainly associated with chronic plaque psoriasis. The dichotomy of IL-26 and IL-17 expression was confirmed in T cells infiltrating psoriatic skin lesions and circulating in the blood where IL-26 producing T cells and IL-17-producing T cells represented distinct cell populations within CXCR3– CCR4+ CCR6+ Th17 cells. Finally, we assessed the pathogenic role of IL-26 in psoriasis by using IL-26-transgenic mice. Skin injury of IL-26 transgenic mice triggered an inflammatory response and a skin phenotype resembling psoriasis, suggesting a pathogenic role in the induction of the disease. Together, our findings indicate that IL-26 is expressed by a distinct subset of Th17 cells in acute forms of psoriasis, where it may drive the inflammatory process and disease development.

5th World Congress scleroderma

Scleroderma The 5th World Congress will be in Bordeaux from 15 to 18 February 2018.

Affiche sclérodermie

Conference Antonin Marchais invited by C. Grosset

Conference FR Transbiomed.
Antonin Marchais, invited by Christophe Grosset.
Conference room of IBGC, 11h

“Characterization of phased siRNA biogenesis in mammals and plants from deep-sequencing data”
Antonin Marchais, Département de cancérologie de l’enfant et l’adolescent. Equipe SPIAJA – Institut Gustave Roussy

Over the past few years, important new aspects of small RNA biogenesis, maturation, editing and circulation have been described. These sophisticated processes vary with biological context and produce small RNAs with a wide range of sequence and spatial distributions. Statistical analysis of these variations from deep-sequencing data enables to identify the triggering biological perturbations; even at long distance from the initiator tissue if applied to circulating small RNAs.
In this scientific context, I will present our implementation of a versatile tool to detect and study phased biogenesis of viral derived siRNAs in mammals and endogenous siRNAs in Plants. Second half of the seminar will focus about our last work concerning new family of pseudogene derived siRNAs in Plants; their expression, tissue specificity and their probable role as translational repressor.

2017

ICCP 2017

The International porphyrias & Porphyrins Congress will be in Bordeaux from 25 to 28 June 2017

ICCP 2017

2016

6th Congress Autophagy BORDEAUX - 2016 - CFATG

CFATG

ANNUAL CONGRESS OF RESEARCH DERMATOLOGIQUE

ANNUAL CONGRESS OF RESEARCH DERMATOLOGIQUE : CARD Paris

6 and 7 December 2016, PALAIS DES CONGRÈS.

Vitiligo International Symposium

Vis 2016

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2nd Workshop of Regenerative Medicine, Bordeaux 24-26 October 2016

Affiche Congres Medecine regenerative

ESDR 2019

Bordeaux has been chosen to organize the 2019 edition of the European Society for Dermatological Research (ESDR). This annual congress gathers around 1000 investigative dermatologists and researchers, mostly from Europe, but a strong American and Asian participation is expected. Before Bordeaux, Munich (2016), Salzburg (2017), and Orlando (international Investigative Dermatology IID) will be hosting the meeting. The local organizing committee will start preparing this prestigious event very soon.

46th annual ESDR meeting

Munich, Germany
7-10 September 2016

The 46th Annual ESDR Meeting will be held at the Technical University campus in Garching, a short distance from the centre of Munich. The meeting has its own website at.

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