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Kimberly Straley
Researcher at Agios Pharmaceuticals
Publications - 33
Citations - 6055
Kimberly Straley is an academic researcher from Agios Pharmaceuticals. The author has contributed to research in topics: Isocitrate dehydrogenase & IDH1. The author has an hindex of 23, co-authored 33 publications receiving 5193 citations. Previous affiliations of Kimberly Straley include Vertex Pharmaceuticals.
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Journal ArticleDOI
Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809
Fredrick Van Goor,Sabine Hadida,Peter D. J. Grootenhuis,B. Burton,Jeffrey H. Stack,Kimberly Straley,Caroline Decker,Mark R. Miller,Mccartney Jason,Eric R. Olson,Jeffrey J. Wine,Raymond A. Frizzell,Melissa A. Ashlock,Paul A. Negulescu +13 more
TL;DR: F508del-CFTR corrected by VX-809 exhibited biochemical and functional characteristics similar to normal CFTR, including biochemical susceptibility to proteolysis, residence time in the plasma membrane, and single-channel open probability.
Journal ArticleDOI
Targeted Inhibition of Mutant IDH2 in Leukemia Cells Induces Cellular Differentiation
Fang Wang,Jeremy Travins,Byron DeLaBarre,Virginie Penard-Lacronique,Virginie Penard-Lacronique,Virginie Penard-Lacronique,Stefanie Schalm,Erica Hansen,Kimberly Straley,Andrew Kernytsky,Wei Liu,Camelia Gliser,Hua Yang,Stefan Gross,Erin Artin,Véronique Saada,Elena Mylonas,Elena Mylonas,Elena Mylonas,Cyril Quivoron,Cyril Quivoron,Cyril Quivoron,Janeta Popovici-Muller,Jeffrey O. Saunders,Francesco G. Salituro,Shunqi Yan,Stuart Murray,Wentao Wei,Yi Gao,Lenny Dang,Marion Dorsch,Sam Agresta,David P. Schenkein,Scott A. Biller,Shinsan M. Su,Stéphane de Botton,Stéphane de Botton,Stéphane de Botton,Katharine E. Yen +38 more
TL;DR: Evidence is provided that inhibitors targeting mutant IDH2/R140Q could have potential applications as a differentiation therapy for cancer, and a small molecule, AGI-6780, that potently and selectively inhibits the tumor-associated mutant IDh2/ R140Q is developed.
Journal ArticleDOI
Frequent Mutation of Isocitrate Dehydrogenase (IDH)1 and IDH2 in Cholangiocarcinoma Identified Through Broad-Based Tumor Genotyping
Darrell R. Borger,Kenneth K. Tanabe,Kenneth C. Fan,Hector U. Lopez,Valeria Fantin,Kimberly Straley,David P. Schenkein,Aram F. Hezel,Marek Ancukiewicz,Hannah M. Liebman,Eunice L. Kwak,Jeffrey W. Clark,David P. Ryan,Vikram Deshpande,Dora Dias-Santagata,Leif W. Ellisen,Andrew X. Zhu,A. John Iafrate +17 more
TL;DR: In an analysis of frozen tissue specimens, IDH1 mutation was associated with highly elevated tissue levels of the enzymatic product 2-hydroxyglutarate, defining a specific metabolic abnormality in this largely incurable type of gastrointestinal cancer and present a potentially new target for therapy.
Journal ArticleDOI
Rescue of ΔF508-CFTR trafficking and gating in human cystic fibrosis airway primary cultures by small molecules
Fredrick Van Goor,Kimberly Straley,Dong Cao,Jesus E. Gonzalez,Sabine Hadida,Anna R. Hazlewood,John Joubran,Tom Knapp,Lewis R. Makings,Mark Miller,Timothy Neuberger,Eric R. Olson,Victor Panchenko,James A. Rader,Ashvani K. Singh,Jeffrey H. Stack,Roger D. Tung,Peter D. J. Grootenhuis,Paul A. Negulescu +18 more
TL;DR: Two classes of novel, potent small molecules identified from screening compound libraries that restore the function of DeltaF508-CFTR in both recombinant cells and cultures of human bronchial epithelia isolated from CF patients are described.
Journal ArticleDOI
Mutant IDH inhibits HNF-4α to block hepatocyte differentiation and promote biliary cancer
Supriya K. Saha,Christine A. Parachoniak,Krishna S. Ghanta,Julien Fitamant,Kenneth N. Ross,Mortada S. Najem,Sushma Gurumurthy,Esra A. Akbay,Daniela Sia,Daniela Sia,Helena Cornella,Oriana Miltiadous,Chad Walesky,Vikram Deshpande,Andrew X. Zhu,Aram F. Hezel,Katharine E. Yen,Kimberly Straley,Jeremy Travins,Janeta Popovici-Muller,Camelia Gliser,Cristina R. Ferrone,Udayan Apte,Josep M. Llovet,Kwok-Kin Wong,Sridhar Ramaswamy,Sridhar Ramaswamy,Nabeel Bardeesy +27 more
TL;DR: It is shown that mutant IDH blocks liver progenitor cells from undergoing hepatocyte differentiation through the production of 2HG and suppression of HNF-4α, a master regulator of hepatocyte identity and quiescence, and presents a novel genetically engineered mouse model of IDH-driven malignancy.