EN DE

Mr RUFFENACH Frank

Fiche personne

coordonnées

IGBMC
Proteomic Platform
1 rue Laurent Fries
BP 10142
67404 ILLKIRCH

rf4@igbmc.fr

Territoire

Alsace

Statut

Ingénieur/Technicien

affiliation

IGBMC - Institut de génétique et de biologie moléculaire et cellulaire (IGBMC)

équipes/plateformes

Plateforme Plateforme de protéomique et spectrométrie de masse de l'IGBMC

Recherche

Expertises :
- Plateforme:Protéomique

Publications

TAF8 regions important for TFIID lobe B assembly or for TAF2 interactions are required for embryonic stem cell survival.

Scheer E, Luo J, Bernardini A, Ruffenach F, Garnier JM, Kolb-Cheynel I, Gupta K, Berger I, Ranish J, Tora L

J Biol Chem. 2021 Oct 8;:101288

Translation of GGC repeat expansions into a toxic polyglycine protein in NIID defines a novel class of human genetic disorders: the polyG diseases.

Boivin M, Deng J, Pfister V, Grandgirard E, Oulad-Abdelghani M, Morlet B, Ruffenach F, Negroni L, Koebel P, Jacob H, Riet F, Dijkstra AA, McFadden K, Clayton WA, Hong D, Miyahara H, Iwasaki Y, Sone J, Wang Z, Charlet-Berguerand N

Neuron. 2021 Apr 13;:

FFAT motif phosphorylation controls formation and lipid transfer function of inter-organelle contacts.

Di Mattia T, Martinet A, Ikhlef S, McEwen AG, Nominé Y, Wendling C, Poussin-Courmontagne P, Voilquin L, Eberling P, Ruffenach F, Cavarelli J, Slee J, Levine TP, Drin G, Tomasetto C, Alpy F

EMBO J. 2020 Oct 30;:e104369

Reduced autophagy upon C9ORF72 loss synergizes with dipeptide repeat protein toxicity in G4C2 repeat expansion disorders.

Boivin M, Pfister V, Gaucherot A, Ruffenach F, Negroni L, Sellier C, Charlet-Berguerand N

EMBO J.. 2020 Jan 13;:e100574

SynAggreg: A Multifunctional High-Throughput Technology for Precision Study of Amyloid Aggregation and Systematic Discovery of Synergistic Inhibitor Compounds.

Aviolat H, Nominé Y, Gioria S, Bonhoure A, Hoffmann D, Ruhlmann C, Nierengarten H, Ruffenach F, Villa P, Trottier Y, Klein FAC

J. Mol. Biol.. 2018 Sep 25;:

rbFOX1/MBNL1 competition for CCUG RNA repeats binding contributes to myotonic dystrophy type 1/type 2 differences.

Sellier C, Cerro-Herreros E, Blatter M, Freyermuth F, Gaucherot A, Ruffenach F, Sarkar P, Puymirat J, Udd B, Day JW, Meola G, Bassez G, Fujimura H, Takahashi MP, Schoser B, Furling D, Artero R, Allain FHT, Llamusi B, Charlet-Berguerand N

Nat Commun. 2018 05 22;9(1):2009

Translation of Expanded CGG Repeats into FMRpolyG Is Pathogenic and May Contribute to Fragile X Tremor Ataxia Syndrome.

Sellier C, Buijsen RA, He F, Natla S, Jung L, Tropel P, Gaucherot A, Jacobs H, Meziane H, Vincent A, Champy MF, Sorg T, Pavlovic G, Wattenhofer-Donze M, Birling MC, Oulad-Abdelghani M, Eberling P, Ruffenach F, Joint M, Anheim M, Martinez-Cerdeno V, Tassone F, Willemsen R, Hukema RK, Viville S, Martinat C, Todd PK, Charlet-Berguerand N

Neuron. 2017 Jan;93(2):331-347

Loss of C9ORF72 impairs autophagy and synergizes with polyQ Ataxin-2 to induce motor neuron dysfunction and cell death.

Sellier C, Campanari ML, Julie Corbier C, Gaucherot A, Kolb-Cheynel I, Oulad-Abdelghani M, Ruffenach F, Page A, Ciura S, Kabashi E, Charlet-Berguerand N

EMBO J.. 2016 06 15;35(12):1276-97

Sequestration of DROSHA and DGCR8 by expanded CGG RNA repeats alters microRNA processing in fragile X-associated tremor/ataxia syndrome.

Sellier C, Freyermuth F, Tabet R, Tran T, He F, Ruffenach F, Alunni V, Moine H, Thibault C, Page A, Tassone F, Willemsen R, Disney MD, Hagerman PJ, Todd PK, Charlet-Berguerand N

Cell Rep. 2013 Mar 28;3(3):869-80