A5018882222- interferon and immune responses
- Inflammasome and immune disorders
- Systemic Lupus Erythematosus Research
- Immune Response and Inflammation
- Immune Cell Function and Interaction
- Protein Tyrosine Phosphatases
- Cytokine Signaling Pathways and Interactions
- Galectins and Cancer Biology
- Platelet Disorders and Treatments
- T-cell and B-cell Immunology
- Renal Diseases and Glomerulopathies
- Lysosomal Storage Disorders Research
- Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
- Cell Adhesion Molecules Research
- Viral Infections and Vectors
- Atherosclerosis and Cardiovascular Diseases
- Chronic Lymphocytic Leukemia Research
- Multiple Myeloma Research and Treatments
- Monoclonal and Polyclonal Antibodies Research
- NF-κB Signaling Pathways
- CAR-T cell therapy research
- Acute Myeloid Leukemia Research
- Respiratory viral infections research
- RNA regulation and disease
Inserm
2012-2023
Institut Curie
2017-2023
Délégation Paris 5
2014-2020
Sorbonne Paris Cité
2014-2020
Institut des Maladies Génétiques Imagine
2012-2020
Université Paris Cité
2014-2020
Université Paris Sciences et Lettres
2017-2020
Immunité et Cancer
2017-2020
Immunologie et Neurogénétique Expérimentales et Moléculaires
2015
Institut Necker Enfants Malades
2013
Innate immunity to viral infection involves induction of the type I IFN response; however, dysfunctional regulation this pathway leads inappropriate inflammation. Here, we evaluated a nonconsanguineous family mixed European descent, with 4 members affected by systemic inflammatory and autoimmune conditions, including lupus, variable clinical expression. We identified germline dominant gain-of-function mutation in TMEM173, which encodes stimulator gene (STING), individuals. STING is key...
Activation of the cyclic dinucleotide sensor stimulator interferon (IFN) genes (STING) is critical for IFN and inflammatory gene expression during innate immune responses. However, role STING in adaptive immunity still unknown. In this study, we show that activation reduces proliferation T lymphocytes. This activity was independent TBK1 IRF3 recruitment type I but required a distinct C-terminal domain activates NF-κB. Inhibition cell by its relocalization to Golgi apparatus caused mitotic...
The type I interferonopathies comprise a recently recognized group of mendelian diseases characterized by an upregulation interferon signaling. These monogenic phenotypes include classic Aicardi-Goutières syndrome and syndromic forms systemic lupus erythematosus, including familial chilblain spondyloenchondrodysplasia. Dermatologic features provide major diagnostic clue to this disease grouping, as exemplified the described stimulator genes-associated vasculopathy with onset in infancy...
In innate immune cells, intracellular sensors such as cGAS-STING stimulate type I/III interferon (IFN) expression, which promotes antiviral defense and activation. However, how IFN-I/III expression is controlled in adaptive cells poorly understood. Here, we identify a transcriptional rheostat orchestrated by RELA that confers human T with innate-like abilities to produce IFN-I/III. Despite intact signaling, responses are stunted CD4+ compared dendritic or macrophages. We find lysine residues...
Abstract Systemic Lupus Erythematosus (SLE) is an autoimmune and inflammatory disease characterized by uncontrolled production of autoantibodies cytokines such as the type-I interferons. Due to lack precise pathophysiological mechanisms, treatments are based on broad unspecific immunossupression. To identify genetic factors associated with SLE we performed whole exome sequencing identified two RELA heterozygous activating mutations in 3 early-onset familial cases. The corresponding RELA/p65...
RASopathies (Noonan syndrome (NS) and Noonan-related syndromes) are neurodevelopmental syndromes resulting from germline mutations in genes that participate the rat sarcoma/mitogen-activated protein kinases (RAS/MAPK) pathway (PTPN11, SOS1, RAF, KRAS or NRAS SHOC2). Some monogenic conditions associated with development of systemic lupus erythematosus (SLE), a few reports described association SLE, NS.