Institut des Cellules souches pour le traitement et l'étude des maladies monogéniques

Présentation du laboratoire

L'Institut des cellules Souches pour le Traitement et l'Etude des maladies Monogéniques (I-Stem) est un centre de R&D dédié à l'élaboration de traitements fondés sur les potentiels offerts par les cellules souches pluripotentes et applicable aux maladies rares d'origine génétique. Son domaine d'activité s'étend depuis la recherche fondamentale de mécanismes pathologiques jusqu'au transfert de nouvelles thérapies à la recherche clinique. Les grandes indications pathologiques étudiées actuellement sont relatives aux maladies du muscle, des motoneurones et de la peau.

Les 20 dernières publications

Titre Auteurs Date de publication Source
Dual Blockade of Misfolded Alpha-Sarcoglycan Degradation by Bortezomib and Givinostat Combination Lucile Hoch, Celine Bruge, Manon Benabides, Emilie Pellier, Johana Tournois, Gurvan Mahé, Xavier Nissan 27/04/2022 Frontiers in Pharmacology
Muscle cells of sporadic amyotrophic lateral sclerosis patients secrete neurotoxic vesicles Cécile Martinat 01/04/2022 Journal of Cachexia, Sarcopenia and Muscle
Generation of a heterozygous SCN5A knockout human induced pluripotent stem cell line by CRISPR/Cas9 edition Lina El Kassar, Karine Giraud-Triboult 01/04/2022 Stem Cell Research
Clinical Grade Human Pluripotent Stem Cell-Derived Engineered Skin Substitutes Promote Keratinocytes Wound Closure In Vitro Raif Eren Ayata, Gilles Lemaitre, Christine Baldeschi 01/04/2022 Cells
Human-Induced Pluripotent Stem Cell‒Derived Keratinocytes, a Useful Model to Identify and Explore the Pathological Phenotype of Epidermolysis Bullosa Simplex Manon Bonnette, Sabrina Martineau, Nathalie Holic, Gilles Lemaitre, Cécile Martinat, Christine Baldeschi 01/01/2022 Journal of Investigative Dermatology
AAV2/9-mediated silencing of PMP22 prevents the development of pathological features in a rat model of Charcot-Marie-Tooth disease 1 A Patrick Aubourg 01/12/2021 Nature Communications
Optogenetically controlled human functional motor endplate for testing botulinum neurotoxins Juliette Duchesne de Lamotte, Jérôme Polentes, Florine Roussange, Léa Lesueur, Pauline Feurgard, Anselme L. Perrier, Cécile Martinat 01/12/2021 Stem Cell Research and Therapy
Contribution of human pluripotent stem cell-based models to drug discovery for neurological disorders Alexandra Benchoua, Marie Lasbareilles, Johana Tournois 01/12/2021 Cells
Human iPSC-derived neurons reveal early developmental alteration of neurite outgrowth in the late-occurring neurodegenerative Wolfram syndrome Sandra Pourtoy-Brasselet, Axel Sciauvaud, Maria Gabriela Boza-Moran, Michel Cailleret, Margot Jarrige, Hélène Polvèche, Jérôme Polentes, Cécile Martinat, Marc Peschanski, Laetitia Aubry 04/11/2021 American Journal of Human Genetics
Dual imaging of dendritic spines and mitochondria in vivo reveals hotspots of plasticity and metabolic adaptation to stress Nicolas Tricaud 01/11/2021 Neurobiology of Stress
Exosomes: Multi-faceted natural intercellular messengers as new candidates for the treatment of myopathies? Judith Lorant 01/11/2021 Medecine/sciences
Modulation of alternative splicing during early infection of human primary B lymphocytes with Epstein-Barr virus (EBV): a novel function for the viral EBNA-LP protein Hélène Polvèche 11/10/2021 Nucleic Acids Research
Chimeric embryos and pseudo-embryos: An alternative to human embryos for research Cécile Martinat 01/08/2021 Medecine/sciences
Emerging opportunities in human pluripotent stem-cells based assays to explore the diversity of botulinum neurotoxins as future therapeutics Juliette Duchesne de Lamotte, Anselme L. Perrier, Cécile Martinat 02/07/2021 International Journal of Molecular Sciences
Hutchinson-Gilford progeria syndrome: Rejuvenating old drugs to fight accelerated ageing Solenn M. Guilbert, Xavier Nissan 01/06/2021 Methods
Integrative Cell Type-Specific Multi-Omics Approaches Reveal Impaired Programs of Glial Cell Differentiation in Mouse Culture Models of DM1 Hélène Polvèche 05/05/2021 Frontiers in Cellular Neuroscience
Human cytomegalovirus infection is associated with increased expression of the lissencephaly gene PAFAH1B1 encoding LIS1 in neural stem cells and congenitally infected brains Alexandra Benchoua 01/05/2021 Journal of Pathology
Increasing brain palmitoylation rescues behavior and neuropathology in Huntington disease mice Morgane Louessard, Patricia Lino, Anselme L. Perrier 01/03/2021 Science advances
Dynamic extrinsic pacing of the HOX clock in human axial progenitors controls motor neuron subtype specification Hynek Wichterle 01/03/2021 Development (Cambridge)
Myogenesis modelled by human pluripotent stem cells: a multi-omic study of Duchenne myopathy early onset Virginie Mournetas, Emmanuelle Massouridès, Jean Baptiste Dupont, Hélène Polvèche, Margot Jarrige, Alan R.L. Dorval, Christian Pinset 01/02/2021 Journal of Cachexia, Sarcopenia and Muscle

Nombre de publications du laboratoire par domaine scientifique

Chaque publication du laboratoire peut être rangée dans une ou plusieurs disciplines scientifiques : la figure ci-dessus présente le nombre de publications du laboratoire pour chaque discipline de la classification ASJC (Elsevier)