Publications related to current research projects

2021

• A. Bamia, M. Sinane, R. Naït-Saïdi, J. Dhiab, M. Keruzoré, PH. Nguyen, A. Bertho, F. Soubigou, S. Halliez, M. Blondel, C. Trollet, M. Simonelig, G. Friocourt, V. Béringue, F. Bihel, C. Voisset. (2021). Anti-prion drugs targeting the protein folding activity of the ribosome reduce PABPN1 aggregation. Neurotherapeutics 2021, 18(2):1137-1150.
• O. Billant, G. Friocourt, P. Roux, C. Voisset. (2021). p53, A Victim of the Prion Fashion. Cancers (Basel). 13(2):E269.

2020

• Marguet F, Friocourt G, Brosolo M, Sauvestre F, Marcorelles P, Lesueur C, Marret S, Gonzalez BJ, Laquerrière A. (2020). Prenatal alcohol exposure is a leading cause of interneuronopathy in humans. Acta Neuropathol Commun. 8(1):208.
• Zuhra K, Panagaki T, Randi EB, Augsburger F, Blondel M, Friocourt G, Herault Y, Szabo C. (2020). Mechanism of cystathionine-β-synthase inhibition by disulfiram: The role of bis(N,N-diethyldithiocarbamate)-copper(II). Biochem Pharmacol. 182:114267.

2019

• Marechal D, Brault V, Leon A, Martin D, Pereira PL, Loaëc N, Birling MC, Friocourt G, Blondel M, Herault Y. (2019) Cbs overdosage is necessary and sufficient to induce cognitive phenotypes in mouse models of Down syndrome and interacts genetically with Dyrk1a. Hum Mol Genet. doi: 10.1093/hmg/ddy447.
• Malerba A, Roth F, Harish P, Dhiab J, Lu-Nguyen N, Cappellari O, Jarmin S, Mahoudeau A, Ythier V, Lainé J, Negroni E, Abgueguen E, Simonelig M, Guedat P, Mouly V, Butler-Browne G, Voisset C, Dickson G, Trollet C. (2019) Pharmacological modulation of the ER stress response ameliorates oculopharyngeal muscular dystrophy. Hum Mol Genet., doi: 10.1093/hmg/ddz007.

2018

• Curie A*, Friocourt G*, des Portes V, Roy A, Nazir T, Brun A, Cheylus A, Marcorelles P, Retzepi K, Maleki N, Bussy G, Paulignan Y, Reboul A, Ibarrola D, Kong J, Hadjikhani N, Laquerrière A and Gollub R. (2018). Basal ganglia involvement in ARX gene mutated patients: the reason for very specific grasping in ARX mutated patients? Neuroimage Clin. 19:454-465.
• Dubos A, Meziane H, Iacono G, Curie A, Riet F, Martin C, Loaëc N, Birling MC, Selloum M, Normand E, Pavlovic G, Sorg T, Stunnenberg H, Chelly J, Humeau Y, Friocourt G*, Hérault Y* (2018). A new mouse model of ARX dup24 recapitulates the patients’ behavioral and fine motor alterations. Hum Mol Genet. 27(12):2138-2153.

2017

• Billant O, Blondel M, Voisset C. (2017). p53, p63 and p73 in the wonderland of S. cerevisiae. Oncotarget. 8(34): 57855-57869.
• Voisset C, Blondel M, Jones GW, Friocourt G, Stahl G, Chédin S, Béringue V, Gillet R. (2017). The double life of the ribosome: When its protein folding activity supports prion propagation. Prion. 11(2): 89-97.

2016

• Mattiske T, Lee K, Gecz J, Friocourt G, Shoubridge C (2016). Embryonic forebrain transcriptome of mice with polyalanine expansion mutations in the ARX homeobox gene. Hum Mol Genet. 25(24): 5433-5443.
• Blondel M, Soubigou F, Evrard J, Nguyen PH, Hasin N, Chédin S, Gillet R, Contesse MA, Friocourt G, Stahl G, Jones GW, Voisset C (2016). Protein Folding Activity of the Ribosome is involved in Yeast Prion Propagation. Sci Rep. 6: 32117.
• Billant O, Léon A, Le Guellec S, Friocourt G, Blondel M, Voisset C (2016). The dominant-negative interplay between p53, p63 and p73: A family affair. Oncotarget. 7(43): 69549-69564.

2014

• Voisset C, Blondel M. (2014) Chemobiology at happy hour: yeast as a model for pharmacological screening. Med Sci (Paris). 30(12): 1161-1168.
• Marcorelles P, Friocourt G, Uguen A, Ledé F, Férec C, Laquerrière A (2014). Cystic Fibrosis Transmembrane Conductance Regulator Protein (CFTR) Expression in the Developing Human Brain: Comparative Immunohistochemical Study between Patients with Normal and Mutated CFTR. J Histochem Cytochem. 62(11): 791-801.
• Nguyen PH, Hammoud H, Halliez S, Pang Y, Evrard J, Schmitt M, Oumata N, Bourguignon JJ, Sanyal S, Beringue V, Blondel M, Bihel F, Voisset C. (2014). Structure-Activity Relationship Study around Guanabenz Identifies Two Derivatives Retaining Antiprion Activity but Having Lost α2-Adrenergic Receptor Agonistic Activity. ACS Chem Neurosci. 5(10): 1075-82.
• Nguyen PH, Oumata N, Soubigou F, Evrard J, Desban N, Lemoine P, Bouaziz S, Galons H, Blondel M and Voisset C (2014). Evaluation of the antiprion activity of 6-aminophenanthridines and related heterocycles. European Journal of Medicinal Chemistry. 82: 363-371.
• Voisset C, García-Rodríguez N, Birkmire A, Blondel M, Wellinger RE (2014). Using yeast to model calcium-related diseases: Example of the Hailey-Hailey disease. Biochim Biophys Acta, Molecular Cell Research 1843: 2315–2321.

2013

• Quéméner-Redon S, Bénech C, Audebert-Bellanger S, Friocourt G, Planes M, Parent P, Férec C (2013). A small de novo 16q24.1 duplication in a woman with severe clinical features. Eur J Med Genet 56(4): 211-5.
• Pang Y, Kurella S, Voisset C, Samanta D, Banerjee D, Schabe A, Das Gupta C, Galons H, Blondel M, Sanyal S (2013). The antiprion compound 6-aminophenanthridine inhibits the protein folding activity of the ribosome by direct competition. J Biol Chem 288(26): 19081-9.
• Oumata N, Nguyen PH, Beringue V, Soubigou F, Pang Y, Desban N, Massacrier C, Morel Y, Paturel C, Contesse MA, Bouaziz S, Sanyal S, Galons H, Blondel M, Voisset C (2013). The Toll-Like Receptor Agonist Imiquimod Is Active against Prions. PLoS One 8(8):e72112.

2012

• Marcorelles P, Gillet D, Friocourt G, Ledé F, Samaison L, Huguen G and Férec C (2012). CFTR protein expression in the male excretory duct system during development. Hum Pathol 43: 390-397.

2011

• Reis SD, Pang Y, Vishnu N, Voisset C, Galons H, Blondel M and Sanyal S (2011). Mode of action of the antiprion drugs 6AP and GA on ribosome assisted protein folding. Biochimie 93: 1047-1054.
• Voisset C, Saupe SJ and Blondel M (2011). The various facets of the protein-folding activity of the ribosome. Biotechnol J 6: 668-673.
• Barbezier N, Chartier A, Bidet Y, Buttstedt A, Voisset C, Galons H, Blondel M, Schwarz E and Simonelig M (2011). Antiprion drugs 6-aminophenanthridine and guanabenz reduce PABPN1 toxicity and aggregation in oculopharyngeal muscular dystrophy. EMBO Mol Med 3: 35-49.
• Quillé ML, Carat S, Quéméner-Redon S, Hirchaud E, Baron D, Benech C, Guihot J, Placet M, Mignen O, Férec C, Houlgatte R and Friocourt G (2011). High-throughput analysis of promoter occupancy reveals new targets for Arx, a gene mutated in mental retardation and interneuronopathies. PLoS One 6: e25181.
• Friocourt G, Marcorelles P, Saugier-Veber P, Quille ML, Marret S and Laquerrière A (2011). Role of cytoskeletal abnormalities in the neuropathology and pathophysiology of type I lissencephaly. Acta Neuropathol 121: 149-170.
• Friocourt G and Parnavelas JG (2011). Identification of Arx targets unveils new candidates for controlling cortical interneuron migration and differentiation. Front Cell Neurosci 5: 28.

2010

• Gug F, Oumata N, Tribouillard-Tanvier D, Voisset C, Desban N, Bach S, Blondel M, and Galons H (2010). Synthesis of conjugates of 6-aminophenanthridine and guanabenz, two structurally unrelated prion inhibitors, for the determination of their cellular targets by affinity chromatography. Bioconjug Chem 21: 279-288.
• Lecourtois M, Poirier K, Friocourt G, Jaglin X, Goldenberg A, Saugier-Veber P, Chelly J and Laquerrière A. (2010). Human lissencephaly with cerebellar hypoplasia due to mutations in TUBA1A: expansion of the foetal neuropathological phenotype. Acta Neuropathol 119: 779-789.
• Marcorelles P, Laquerrière A, Adde-Michel C, Marret S, Saugier-Veber P, Beldjord C and Friocourt G (2010). Evidence for tangential migration disturbances in human lissencephaly resulting from a defect in LIS1, DCX and ARX genes. Acta Neuropathol 120: 503-515.
• Friocourt G and Parnavelas JG. (2010). Mutations in ARX result in several defects involving GABAergic neurons. Front Cell Neurosci 4: 4.

2009

• Voisset C, Saupe S and Blondel M (2009). Procedure for identification and characterization of drugs efficient against mammalian prion: from a yeast-based antiprion drug screening assay to in vivo mouse models. Infectious Diseases, Drug Targets 9: 31-39.

2008

• Tribouillard-Tanvier D, Beringue V, Desban N, Gug F, Bach S, Voisset C, Galons H, Laude H, Vilette D and Blondel M. (2008). Antihypertensive drug guanabenz is active in vivo against both yeast and mammalian prions. PLoS One 3: e1981.
• Tribouillard-Tanvier D, Dos Reis S, Gug F, Voisset C, Beringue V, Sabate R, Kikovska E, Talarek N, Bach S, Huang C, Desban N, Saupe SJ, Supattapone S, Thuret JY, Chedin S, Vilette D, Galons H, Sanyal S and Blondel M (2008). Protein folding activity of ribosomal RNA is a selective target of two unrelated antiprion drugs. PLoS One 3: e2174.
• Voisset C, Thuret JY, Tribouillard-Tanvier D, Saupe SJ and Blondel M (2008). Tools for the study of ribosome-borne protein folding activity. Biotechnol J 3: 1033-1040.
• Friocourt G, Kanatani S, Tabata H, Yozu M, Takahashi T, Antypa M, Raguénès O, Chelly J, Férec C, Nakajima K, Parnavelas JG (2008). Cell-autonomous roles of ARX in cell proliferation and neuronal migration during corticogenesis. J Neurosci 28: 5794-5805.

2007

• Friocourt G, Liu J, Antypa M, Rakic S, Walsh CA and Parnavelas JG (2007). Both Doublecortin and Doublecortin-like kinase play a role in cortical interneuron migration. J Neurosci 27: 3875-3883.
• Trouvé P, Le Drévo MA, Kerbiriou M, Friocourt G, Fichou Y, Gillet D, Férec C (2007). Annexin V interacts with cystic fibrosis transmembrane conductance regulator. Biochim Biophys Acta 1772: 1121-1133.
• Friocourt G (2007). From genes to human diseases in cortical development. Bioessays 29: 706-709.

2006

• Friocourt G, Poirier K, Rakic S, Parnavelas JG and Chelly J. (2006). The role of ARX in brain development. Eur. J. Neurosci. 23, 869-876.

2005

• Friocourt G, Kappeler C, Saillour Y, Fauchereau F, Rodriguez MS, Bahi N, Vinet MC, Chafey P, Poirier K, Taya, S, Wood, SA, Dargemont C, Francis F and Chelly J. (2005) Doublecortin interacts with the ubiquitin protease DFFRX, which associates with microtubules in neuronal processes. Mol Cell Neurosci 28: 153-164.

2004

• Poirier K, Van Esch H, Friocourt G, Saillour Y, Bahi N, Backer S, Souil E, Castelnau-Ptakhine L, Beldjord C, Francis F, Bienvenu T and Chelly J (2004). Neuroanatomical distribution of ARX in brain and its localisation in GABAergic neurons. Mol Brain Res 122: 35-46.

2003

• Bahi N, Friocourt G, Carrié A, Graham ME, Weiss JL, Chafey P, Fauchereau F, Burgoyne RD and Chelly J (2003). IL1 receptor accessory protein like, a protein involved in X-linked mental retardation, interacts with Neuronal Calcium Sensor-1 and regulates exocytosis. Hum Mol Genet 12: 1415-25.
• Friocourt G, Koulakoff A, Chafey P, Boucher D, Fauchereau F, Chelly J and Francis F (2003). Doublecortin functions at the extremities of growing neuronal processes. Cereb Cortex 13: 620-626.

2002

• Bienvenu T, Poirier K, Friocourt G, Bahi N, Beaumont D, Fauchereau F, Ben Jeema L, Zemni R, Vinet MC, Francis F, Couvert P, Gomot M, Moraine C, van Bokhoven H, Kalscheuer V, Frints S, Gecz J, Ohzaki K, Chaabouni H, Fryns JP, Des Portes V, Beldjord C, and Chelly J (2002). ARX, a novel Prd-class-homeobox gene highly expressed in the telencephalon, is mutated in X-linked mental retardation. Hum Mol Genet 11: 981-991.
• Benchoua A, Couriaud C, Guegan C, Tartier L, Couvert P, Friocourt G, Chelly J, Menissier-De Murcia J and Onteniente B. (2002). Active caspase-8 translocates into the nucleus of apoptotic cells to inactivate poly(ADP-ribose) polymerase-2. J Biol Chem 277: 34217-22.

2001

• Bordet T, Castelnau-Ptakhine L, Fauchereau F, Friocourt G, Kahn A and Haase G. (2001). Neuronal targeting of cardiotrophin-1 by coupling with tetanus toxin C fragment. Mol Cell Neurosci 17: 842-854.
• Friocourt G, Chafey P, Billuart P, Koulakoff A, Vinet MC, Schaar BT, McConnell SK, Francis F and Chelly J. (2001). Doublecortin interacts with µ Subunits of clathrin adaptor complexes in the developing nervous system. Mol Cell Neurosci 18: 307-319.

2000

• Zemni R, Bienvenu T, Vinet MC, Sefiani A, Carrie A, Billuart P, McDonell N, Couvert P, Francis F, Chafey P, Fauchereau F, Friocourt G, Des Portes V, Cardona A, Frints S, Meindl A, Brandau O, Ronce N, Moraine C, van Bokhoven H, Ropers HH, Sudbrak R, Kahn A, Fryns JP, Beldjord C and Chelly J. (2000). A new gene involved in X-linked mental retardation identified by analysis of an X;2 balanced translocation. Nat Genet 24: 167-170.

1999

• Francis F, Koulakoff A, Boucher D, Chafey P, Schaar B, Vinet MC, Friocourt G, McDonnell N, Reiner O, Kahn A, McConnell SK, Berwald-Netter Y, Denoulet P and Chelly J. (1999). Doublecortin is a developmentally regulated microtubule-associated protein expressed in migrating and differentiating neurons. Neuron 23: 247-256.

Complete liste of publications of C. Voisset

Complete liste of publications of G. Friocourt