2025 iAds video contest for 14-15 years old students
A video contest as part of the iAds project coordinated by the IGMM (Eric Kremer team) has been launched among Montpellier high schools for 14-15 years old students.
For more information, visit the video contest webpage.
Composition of the sub-group: Florence Rage, Johann Soret, Pauline Duc et Audrey Moisan.
Understanding the molecular mechanisms of Spinal Muscular Atrophy (SMA)
INTERDISCIPLINARY MASTER SCHOLARSHIPS – IGMM (cnrs.fr) – IGMM
Spinal Muscular Atrophy (SMA), a hereditary disease, results from degeneration of motor neurons leading to severe muscular atrophy. This disease is caused by the deletion and/or mutation of the SMN1 gene. Due to its crucial role in the biogenesis of spliceosome components, SMN deficiency is correlated to numerous splicing alterations. At the cytoplasmic level, given the high polarity of motor neurons, multiple other molecular mechanisms are impaired in this disease, including mRNA transport along axons of motor neurons and the formation and the maintenance of neuromuscular junctions (NMJs).
In our projects, we are using human induced pluripotent stem cells (hIPSCs) from healthy and SMA individuals to obtain motor neurons and study :
- The splicing defects of mRNAs in SMN deficient cells.
- The localization and local translation of mRNAs along axons of motor neurons using the MS2 and SunTag systems, integrated in target genes by the CRISPR-Cas9 technology.
- The SMN-interacting protein network in control and SMA MNs and myoblasts derived from iPSCs by combining CRISPR-Cas9 technology and biotin proximity-dependent labeling (BioID).
- The formation, development and maintenance of mature and functional human NMJs using microfluidic devices coupled with microelectrode array (MEA) in an interdisciplinary collaboration with Benoît Charlot (IES) and Gilles Carnac (Phymedexp) (Duc et al., 2021 Lab Chip, doi 10.1039/d1lc00497b).
- Finally, in collaboration with Eran Perlson (Tel Aviv University, Israel), we are also developing 24-well plate devices, combined with MEA, in order to recapitulate NMJs from SMA and ALS patients and to perform drug assays with personalized medicine approach.
