4 research departments
750 employees
45 nationalities
55 research teams
16 ERC laureates
260 publications per year
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Fragile-X Syndrome: one more step to finding a cure for this disease

Under normal condition (left), FMRP helps produce the Dgkk enzyme in neurons. In the fragile X situation, in the absence of FMRP (right), Dgkk is not sufficiently produced. Acting on Dgk enzymatic activity would be a way to correct the defects associated with the fragile X syndrome.
 
© IGBMC / Hervé Moine

July 11, 2016

A study supervised by Hervé Moine evidenced a pathogenic mechanism for fragile X syndrome. This genetic disease most often causes intellectual disabilities, behavioral problems and physical abnormalities. These results were published in the Proceedings of the National Academy of Sciences (PNAS) on May 27th.

 

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A breakthrough in our understanding of inflammatory bowel diseases (IBD) and colitis-associated cancer

Schematic drawing illustrating two intestinal epithelial cells (IEC) attached through α6β4 integrin to the basement membrane (BM), their specialized extracellular matrix (ECM). Loss of the integrin impairs epithelial integrity causing epithelium fragility and detachment from the BM. This cellular stress triggers an “alarm signal” in IECs (IL-18). Consequently, the mucus layer becomes altered, favoring bacterial translocation through the epithelial barrier and activation of the immune system. Infection and tissue injury then lead to a strong inflammation, mediated by the massive recruitment of innate immune cells and the secretion of IL-1β. With time, cells of the adaptive immunity sustain and perpetuate chronic inflammation, and ultimately induce the spontaneous development of cancer (CC, cancer cells).

Sept. 14, 2016

The work done by the team of Elisabeth Georges-Labouesse, with the help of Michel Labouesse at the IGBMC, has highlighted a novel and indispensable role for the extracellular matrix (ECM) which anchors cells, similar to foundations which support a house, in protecting the intestine against inflammation and secondary cancer development. These results provide new insights in the understanding of IBD and colitis-associated colorectal cancer and will help pave the way towards the development of novel therapies. This work was published on the 1st of July in the Journal Gut.

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Gene function: solving the puzzle is on the right track

3D imaging can reveal more precisely the complexity of embryonic development in mice with viable solutions to genetic analysis of broadband to understand gene function.

Sept. 22, 2016

As part of the International Mouse Phenotyping Consortium, the French National Infrastructure for mouse phenogenomics, PHENOMIN hosted by the Mouse Clinical Institute (IGBMC, UMR7104 CNRS, INSERM, and University of Strasbourg) has coordinated its efforts with 17 other centers to reveal the important role of almost 1 out of 4 genes for controlling normal development of the mouse embryo. These results were published in Nature on September 22th.

 

 

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