The François Jacob Institute of Biology brings together five departments and three services
The last two years in scientific news
Find our latest scientific news, as well as coming and past seminars and PhD defences.
The term "synucleinopathy" covers a range of diseases, including Parkinson's disease, Lewy body dementia and multiple system atrophy, that involve pathological alpha-synuclein aggregations. An analysis of the properties of the different aggregate strains present in synucleinopathies has delineated the molecular bases of the diseases. The study, led by MIRCen (CEA-Jacob) researchers in partnership with the University of Louvain and Imperial College London, was published in Acta Neuropathologica
Scientists from the Laboratory of Neurodegenerative Diseases (MIRCen/ François Jacob Institute of Biology) in collaboration with the Neurocentre Magendie (Inserm/University of Bordeaux) have highlighted the decisive role played by a metabolic pathway in memory disorders in Alzheimer's disease. Their work, published in Cell Metabolism, also shows that L-serine intake, in the form of a dietary supplement, restores the spatial memory that is affected early on in mouse models of the disease.
As part of an international partnership, a team from the Neurodegenerative Diseases Laboratory (MIRCen) has characterized the pathological effects of various alpha-synuclein aggregate strains in vivo. In their work, published in Acta Neuropathologica Communications, the researchers showed that the conformation of these aggregates plays a key role in their amplification and propagation in the brain.
A new structure for aggregated alpha-synuclein has been determined at the atomic level using cryo-electron microscopy. The work, lead by a team from MIRCen's Neurodegenerative Diseases Laboratory, was published in the journal eLife. The determination of this previously undescribed structure opens new doors for the development of ligands specifically targeting alpha-synuclein aggregates in Parkinson's disease.
The weekly MIRCen laboratory meeting is a particularly important time, at which external collaborators or students from the laboratory present their research projects.
Below is a list of speakers from external laboratories invited to speak at these meetings in the next few months.
If a PhD thesis is to be defended at the CEA at Fontenay-aux-Roses, it is possible to attend provided that a completed form is sent to the MIRCen secretary at least three days in advance.
PhD Student: Marianne Maugard.
Les patients atteints de la maladie d'Alzheimer ont des déficits du métabolisme cérébral du glucose une quinzaine d'années avant les premiers défauts cognitifs, suggérant que le métabolisme pourrait contribuer à la physiopathologie de la MA. Pour mieux comprendre les mécanismes qui relient le métabolisme énergétique et l'activité synaptique, nous nous sommes intéressés à la production de L-serine, une molécule dont la synthèse de novo dérive d'un intermédiaire de la glycolyse.
PhD Student: Séverine Maire.
Huntington’s disease (HD) is a genetic disorder caused by the expansion of a CAG repeat in the first exon of the Huntingtin gene (HTT). We propose to use trans-splicing to develop a gene therapy vector that will significantly reduce or eliminate the expression of the mutant protein while restoring a physiological level of normal HTT in cells affected by the HD mutation.
PhD Student: Jérémy Pépin.
Previous studies have shown that the metabolic profile measured in ¹H NMR spectroscopy can be altered in patients with Huntington's disease. Hypotheses involving defects in energy metabolism have been advanced to explain partially the pathophysiology of the disease. The metabolic actors could thus be biomarkers of interest. All of the results and methods implemented during this thesis show the potential of a promising MRI modality called CEST (Chemical Exchance Saturation Transfer) to identify potential biomarkers of neurodegenerative diseases.
CEA is a French government-funded technological research organisation in four main areas: low-carbon energies, defense and security, information technologies and health technologies. A prominent player in the European Research Area, it is involved in setting up collaborative projects with many partners around the world.