Centre de Référence Maladies Rares


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

FR: Biologie Fondamentale Appliquée à la Médecine

U1035Display navigation news FR / EN


ESDR meeting 2019

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


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

More info :

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.


ICCP 2017

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

ICCP 2017


6th Congress Autophagy BORDEAUX - 2016 - CFATG




6 and 7 December 2016, PALAIS DES CONGRÈS.

Vitiligo International Symposium

Vis 2016


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.



The newly established « AquiDerm Cellule de Transfert » funded in part by Région Aquitaine is building on previously established network with dermocosmetics companies to develop a better valorisation of the know-how of the Dermatology team. In 2014, a technician position has been funded together with an equipment for metabolomics. The plan over 3 years is to create two further full time positions of technician/engineer which a part-time devoted to basic research in academic projects.


Vitiligo is the most common depigmenting disorder, with a prevalence of approximately 1% in the world population. This disease involves complex combinatorial pathogenic effects, multiple susceptibility genes and unknown environmental triggers. A loss of epidermal melanocytes is the pathogenic hallmark of vitiligo. The dermatology team has a long lasting interest in pigmentary disorders especially vitiligo and has developed tools to model pigmented skin ex vivo for the last two decades. The major goal of the newly created ATIP-Avenir project is to develop a basic research program on inflammatory dermatoses, in particular vitiligo, which benefits already of an established clinical research programme.

INSERM U1035! - University of Bordeaux
146 rue Léo Saignat - PE building south zone, 4th floor
33000 Bordeaux
Work +33 557 57 13 73/74