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Randal Kaufman

Title(s)Adjunct Professor, Pharmacology
SchoolHealth Sciences
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    Collapse Research 
    Collapse Research Activities and Funding
    A Novel Role for the UPR Component, ATF6 in AD-associated Neuroprotective Pathways
    NIH/NIA R01AG062190Mar 1, 2019 - Dec 31, 2023
    Role: Principal Investigator
    Mechanism of ER protein misfolding-induced mitochondrial dysfunction
    NIH/NIDDK R01DK113171Sep 18, 2017 - Aug 31, 2021
    Role: Principal Investigator
    Modifiers of Proinsulin Influence T2D Susceptibility
    NIH/NIDDK R24DK110973Sep 12, 2016 - Jun 30, 2020
    Role: Principal Investigator
    ER stress and UPR in non-alcoholic steatohepatitis and hepatocellular carcinoma
    NIH/NCI R01CA198103May 1, 2016 - Apr 30, 2021
    Role: Principal Investigator
    Homeostatic Role of IRE1a-XBP1-PDI1 in Hepatic Lipid Metabolism
    NIH/NIDDK R01DK103185Apr 1, 2015 - Mar 31, 2020
    Role: Principal Investigator
    eIF2a phosphorylation as a novel druggable target in CRPC
    NIH/NCI R21CA190588Jan 5, 2015 - Dec 31, 2016
    Role: Co-Principal Investigator
    Alteration in protein interactions with proinsulin and insulin in type 2 diabetes
    NIH/NIDDK R24DK093074Sep 1, 2011 - Feb 28, 2013
    Role: Principal Investigator
    Regulation of ER stress-induced cell death
    NIH/NIDDK R01DK088227Sep 1, 2010 - May 31, 2015
    Role: Principal Investigator
    HTP chemical genomic screens to identify positive regulators of the Unfolded Prot
    NIH/NIMH R03MH089782Sep 30, 2009 - May 31, 2011
    Role: Principal Investigator
    High Throughput Chemical Genomics to Identify Novel Inhibitors of CHOP
    NIH/NIMH R03MH084182Jun 1, 2008 - May 31, 2009
    Role: Principal Investigator
    Unfolded proteins in the Endoplasmic Reticulum to Disease
    NIH/NIDDK R13DK079785Jul 15, 2007 - Jun 30, 2008
    Role: Principal Investigator
    Conformational Diseases of the Secretory Pathway
    NIH/NIDDK R13DK063935Jan 15, 2003 - Dec 31, 2003
    Role: Principal Investigator
    Role of the Unfolded Protein Response in Beta Cell
    NIH/NIDDK R37DK042394Jan 1, 1998 - Aug 31, 2017
    Role: Principal Investigator
    Unfolded Protein Response in Glucose Homeostasis
    NIH/NIDDK R01DK042394Jan 1, 1998 - Jun 30, 2007
    Role: Principal Investigator
    TRANSLATIONAL CONTROL IN REGULATION OF APOPTOSIS
    NIH/NIAID R01AI042394Jan 1, 1998 - Jun 30, 2003
    Role: Principal Investigator
    Regulation of Factor FVIII Secretion
    NIH/NHLBI R01HL052173Aug 1, 1995 - Jun 30, 2015
    Role: Principal Investigator
    ADENOVIRUS--MEDIATED DELIVERY OF NOVEL FACTOR VIII
    NIH/NHLBI R01HL053777Sep 30, 1994 - Aug 31, 2002
    Role: Principal Investigator
    GENETIC ENGINEERING OF AN IMPROVED FACTOR VIII PRODUCT
    NIH/NHLBI R44HL035946Mar 1, 1988 - Feb 28, 1990
    Role: Principal Investigator
    PRODUCTION OF MONOCLONAL ANTIBODIES IN TRANSGENIC MICE
    NIH/NIGMS R44GM037329Jul 1, 1986 - Aug 31, 1990
    Role: Principal Investigator
    PRODUCTION OF HUMAN MONOCLONAL ANTIBODIES
    NIH/NIGMS R43GM037329Jul 1, 1986 - Dec 31, 1986
    Role: Principal Investigator

    Collapse Bibliographic 
    Collapse Publications
    Publications listed below are automatically derived from MEDLINE/PubMed and other sources, which might result in incorrect or missing publications. Researchers can login to make corrections and additions, or contact us for help.
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    1. Jang I, Pottekat A, Poothong J, Yong J, Lagunas-Acosta J, Charbono A, Chen Z, Scheuner DL, Liu M, Itkin-Ansari P, Arvan P, Kaufman RJ. PDIA1/P4HB is required for efficient proinsulin maturation and ß cell health in response to diet induced obesity. Elife. 2019 Jun 11; 8. PMID: 31184304.
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    2. Arunagiri A, Haataja L, Pottekat A, Pamenan F, Kim S, Zeltser LM, Paton AW, Paton JC, Tsai B, Itkin-Ansari P, Kaufman RJ, Liu M, Arvan P. Proinsulin misfolding is an early event in the progression to type 2 diabetes. Elife. 2019 Jun 11; 8. PMID: 31184302.
      View in: PubMed
    3. Cole DW, Svider PF, Shenouda KG, Lee PB, Yoo NG, McLeod TM, Mutchnick SA, Yoo GH, Kaufman RJ, Callaghan MU, Fribley AM. Targeting the unfolded protein response in head and neck and oral cavity cancers. Exp Cell Res. 2019 May 07. PMID: 31075256.
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    4. Shah O'Brien P, Xi Y, Miller JR, Brownell AL, Zeng Q, Yoo GH, Garshott DM, O'Brien MB, Galinato AE, Cai P, Narula N, Callaghan MU, Kaufman RJ, Fribley AM. Disulfiram (Antabuse) Activates ROS-Dependent ER Stress and Apoptosis in Oral Cavity Squamous Cell Carcinoma. J Clin Med. 2019 May 06; 8(5). PMID: 31064122.
      View in: PubMed
    5. Mimura J, Inose-Maruyama A, Taniuchi S, Kosaka K, Yoshida H, Yamazaki H, Kasai S, Harada N, Kaufman RJ, Oyadomari S, Itoh K. Concomitant Nrf2- and ATF4-activation by Carnosic Acid Cooperatively Induces Expression of Cytoprotective Genes. Int J Mol Sci. 2019 Apr 05; 20(7). PMID: 30959808.
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    6. Li Y, Tinoco R, Elmén L, Segota I, Xian Y, Fujita Y, Sahu A, Zarecki R, Marie K, Feng Y, Khateb A, Frederick DT, Ashkenazi SK, Kim H, Perez EG, Day CP, Segura Muñoz RS, Schmaltz R, Yooseph S, Tam MA, Zhang T, Avitan-Hersh E, Tzur L, Roizman S, Boyango I, Bar-Sela G, Orian A, Kaufman RJ, Bosenberg M, Goding CR, Baaten B, Levesque MP, Dummer R, Brown K, Merlino G, Ruppin E, Flaherty K, Ramer-Tait A, Long T, Peterson SN, Bradley LM, Ronai ZA. Gut microbiota dependent anti-tumor immunity restricts melanoma growth in Rnf5-/- mice. Nat Commun. 2019 04 02; 10(1):1492. PMID: 30940817.
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    7. Li H, O'Meara M, Zhang X, Zhang K, Seyoum B, Yi Z, Kaufman RJ, Monks TJ, Wang JM. Ameliorating Methylglyoxal-Induced Progenitor Cell Dysfunction for Tissue Repair in Diabetes. Diabetes. 2019 Jun; 68(6):1287-1302. PMID: 30885990.
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    8. Zhu H, Bhatt B, Sivaprakasam S, Cai Y, Liu S, Kodeboyina SK, Patel N, Savage NM, Sharma A, Kaufman RJ, Li H, Singh N. Ufbp1 promotes plasma cell development and ER expansion by modulating distinct branches of UPR. Nat Commun. 2019 03 06; 10(1):1084. PMID: 30842412.
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    9. Kim JY, Garcia-Carbonell R, Yamachika S, Zhao P, Dhar D, Loomba R, Kaufman RJ, Saltiel AR, Karin M. ER Stress Drives Lipogenesis and Steatohepatitis via Caspase-2 Activation of S1P. Cell. 2018 Sep 20; 175(1):133-145.e15. PMID: 30220454.
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    10. Liu M, Weiss MA, Arunagiri A, Yong J, Rege N, Sun J, Haataja L, Kaufman RJ, Arvan P. Biosynthesis, structure, and folding of the insulin precursor protein. Diabetes Obes Metab. 2018 09; 20 Suppl 2:28-50. PMID: 30230185.
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    11. Poothong J, Sopha P, Kaufman RJ, Tirasophon W. IRE1a nucleotide sequence cleavage specificity in the unfolded protein response. FEBS Lett. 2018 08; 592(15):2669. PMID: 30168599.
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    12. Pugach EK, Feltes M, Kaufman RJ, Ory DS, Bang AG. High-content screen for modifiers of Niemann-Pick type C disease in patient cells. Hum Mol Genet. 2018 06 15; 27(12):2101-2112. PMID: 29659804.
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    13. Wang JM, Qiu Y, Yang Z, Kim H, Qian Q, Sun Q, Zhang C, Yin L, Fang D, Back SH, Kaufman RJ, Yang L, Zhang K. IRE1a prevents hepatic steatosis by processing and promoting the degradation of select microRNAs. Sci Signal. 2018 May 15; 11(530). PMID: 29764990.
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    14. Chandrahas VK, Han J, Kaufman RJ. Coordinating Organismal Metabolism During Protein Misfolding in the ER Through the Unfolded Protein Response. Curr Top Microbiol Immunol. 2018; 414:103-130. PMID: 28900680.
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    15. Zhang S, Macias-Garcia A, Velazquez J, Paltrinieri E, Kaufman RJ, Chen JJ. HRI coordinates translation by eIF2aP and mTORC1 to mitigate ineffective erythropoiesis in mice during iron deficiency. Blood. 2018 01 25; 131(4):450-461. PMID: 29101239.
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    16. Choi WG, Han J, Kim JH, Kim MJ, Park JW, Song B, Cha HJ, Choi HS, Chung HT, Lee IK, Park TS, Hatzoglou M, Choi HS, Yoo HJ, Kaufman RJ, Back SH. eIF2a phosphorylation is required to prevent hepatocyte death and liver fibrosis in mice challenged with a high fructose diet. Nutr Metab (Lond). 2017; 14:48. PMID: 28781602.
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    17. Han J, Kaufman RJ. Physiological/pathological ramifications of transcription factors in the unfolded protein response. Genes Dev. 2017 07 15; 31(14):1417-1438. PMID: 28860159.
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    18. DeZwaan-McCabe D, Sheldon RD, Gorecki MC, Guo DF, Gansemer ER, Kaufman RJ, Rahmouni K, Gillum MP, Taylor EB, Teesch LM, Rutkowski DT. ER Stress Inhibits Liver Fatty Acid Oxidation while Unmitigated Stress Leads to Anorexia-Induced Lipolysis and Both Liver and Kidney Steatosis. Cell Rep. 2017 05 30; 19(9):1794-1806. PMID: 28564599.
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    19. Poothong J, Tirasophon W, Kaufman RJ. Functional analysis of the mammalian RNA ligase for IRE1 in the unfolded protein response. Biosci Rep. 2017 04 30; 37(2). PMID: 28093457.
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    20. Poothong J, Sopha P, Kaufman RJ, Tirasophon W. IRE1a nucleotide sequence cleavage specificity in the unfolded protein response. FEBS Lett. 2017 01; 591(2):406-414. PMID: 28027394.
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    21. Yao T, Deng Z, Gao Y, Sun J, Kong X, Huang Y, He Z, Xu Y, Chang Y, Yu KJ, Findley BG, Berglund ED, Wang RT, Guo H, Chen H, Li X, Kaufman RJ, Yan J, Liu T, Williams KW. Ire1a in Pomc Neurons Is Required for Thermogenesis and Glycemia. Diabetes. 2017 03; 66(3):663-673. PMID: 28028078.
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    22. Chiang WC, Chan P, Wissinger B, Vincent A, Skorczyk-Werner A, Krawczynski MR, Kaufman RJ, Tsang SH, Héon E, Kohl S, Lin JH. Achromatopsia mutations target sequential steps of ATF6 activation. Proc Natl Acad Sci U S A. 2017 01 10; 114(2):400-405. PMID: 28028229.
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    23. Placzek AN, Prisco GV, Khatiwada S, Sgritta M, Huang W, Krnjevic K, Kaufman RJ, Dani JA, Walter P, Costa-Mattioli M. eIF2a-mediated translational control regulates the persistence of cocaine-induced LTP in midbrain dopamine neurons. Elife. 2016 12 13; 5. PMID: 27960077.
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    24. Jin JK, Blackwood EA, Azizi K, Thuerauf DJ, Fahem AG, Hofmann C, Kaufman RJ, Doroudgar S, Glembotski CC. ATF6 Decreases Myocardial Ischemia/Reperfusion Damage and Links ER Stress and Oxidative Stress Signaling Pathways in the Heart. Circ Res. 2017 Mar 03; 120(5):862-875. PMID: 27932512.
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    25. Oubaha M, Miloudi K, Dejda A, Guber V, Mawambo G, Germain MA, Bourdel G, Popovic N, Rezende FA, Kaufman RJ, Mallette FA, Sapieha P. Senescence-associated secretory phenotype contributes to pathological angiogenesis in retinopathy. Sci Transl Med. 2016 10 26; 8(362):362ra144. PMID: 27797960.
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    26. Bai Y, Kinne J, Donham B, Jiang F, Ding L, Hassler JR, Kaufman RJ. Read-Split-Run: an improved bioinformatics pipeline for identification of genome-wide non-canonical spliced regions using RNA-Seq data. BMC Genomics. 2016 08 22; 17 Suppl 7:503. PMID: 27556805.
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    27. Grootjans J, Kaser A, Kaufman RJ, Blumberg RS. The unfolded protein response in immunity and inflammation. Nat Rev Immunol. 2016 08; 16(8):469-84. PMID: 27346803.
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    28. Yang L, Li S, Miao L, Huang H, Liang F, Teng X, Xu L, Wang Q, Xiao W, Ridder WH, Ferguson TA, Chen DF, Kaufman RJ, Hu Y. Rescue of Glaucomatous Neurodegeneration by Differentially Modulating Neuronal Endoplasmic Reticulum Stress Molecules. J Neurosci. 2016 05 25; 36(21):5891-903. PMID: 27225776.
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    29. Han J, Kaufman RJ. The role of ER stress in lipid metabolism and lipotoxicity. J Lipid Res. 2016 08; 57(8):1329-38. PMID: 27146479.
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    30. Ravindran R, Loebbermann J, Nakaya HI, Khan N, Ma H, Gama L, Machiah DK, Lawson B, Hakimpour P, Wang YC, Li S, Sharma P, Kaufman RJ, Martinez J, Pulendran B. The amino acid sensor GCN2 controls gut inflammation by inhibiting inflammasome activation. Nature. 2016 Mar 24; 531(7595):523-527. PMID: 26982722.
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    31. Wang M, Kaufman RJ. Protein misfolding in the endoplasmic reticulum as a conduit to human disease. Nature. 2016 Jan 21; 529(7586):326-35. PMID: 26791723.
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    32. Yong J, Itkin-Ansari P, Kaufman RJ. When Less Is Better: ER Stress and Beta Cell Proliferation. Dev Cell. 2016 Jan 11; 36(1):4-6. PMID: 26766438.
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    33. Hassler JR, Scheuner DL, Wang S, Han J, Kodali VK, Li P, Nguyen J, George JS, Davis C, Wu SP, Bai Y, Sartor M, Cavalcoli J, Malhi H, Baudouin G, Zhang Y, Yates JR, Itkin-Ansari P, Volkmann N, Kaufman RJ. The IRE1a/XBP1s Pathway Is Essential for the Glucose Response and Protection of ß Cells. PLoS Biol. 2015 10; 13(10):e1002277. PMID: 26469762.
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    34. Sidhu A, Miller JR, Tripathi A, Garshott DM, Brownell AL, Chiego DJ, Arevang C, Zeng Q, Jackson LC, Bechler SA, Callaghan MU, Yoo GH, Sethi S, Lin HS, Callaghan JH, Tamayo-Castillo G, Sherman DH, Kaufman RJ, Fribley AM. Borrelidin Induces the Unfolded Protein Response in Oral Cancer Cells and Chop-Dependent Apoptosis. ACS Med Chem Lett. 2015 Nov 12; 6(11):1122-7. PMID: 26617965.
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    35. Cruz PG, Fribley AM, Miller JR, Larsen MJ, Schultz PJ, Jacob RT, Tamayo-Castillo G, Kaufman RJ, Sherman DH. Novel Lobophorins Inhibit Oral Cancer Cell Growth and Induce Atf4- and Chop-Dependent Cell Death in Murine Fibroblasts. ACS Med Chem Lett. 2015 Aug 13; 6(8):877-81. PMID: 26288688.
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    36. D'Osualdo A, Anania VG, Yu K, Lill JR, Kaufman RJ, Matsuzawa S, Reed JC. Transcription Factor ATF4 Induces NLRP1 Inflammasome Expression during Endoplasmic Reticulum Stress. PLoS One. 2015; 10(6):e0130635. PMID: 26086088.
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    37. Kohl S, Zobor D, Chiang WC, Weisschuh N, Staller J, Gonzalez Menendez I, Chang S, Beck SC, Garcia Garrido M, Sothilingam V, Seeliger MW, Stanzial F, Benedicenti F, Inzana F, Héon E, Vincent A, Beis J, Strom TM, Rudolph G, Roosing S, Hollander AI, Cremers FP, Lopez I, Ren H, Moore AT, Webster AR, Michaelides M, Koenekoop RK, Zrenner E, Kaufman RJ, Tsang SH, Wissinger B, Lin JH. Mutations in the unfolded protein response regulator ATF6 cause the cone dysfunction disorder achromatopsia. Nat Genet. 2015 Jul; 47(7):757-65. PMID: 26029869; PMCID: PMC4610820.
    38. Zhou AX, Wang X, Lin CS, Han J, Yong J, Nadolski MJ, Borén J, Kaufman RJ, Tabas I. C/EBP-Homologous Protein (CHOP) in Vascular Smooth Muscle Cells Regulates Their Proliferation in Aortic Explants and Atherosclerotic Lesions. Circ Res. 2015 May 22; 116(11):1736-43. PMID: 25872946.
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    39. Han J, Song B, Kim J, Kodali VK, Pottekat A, Wang M, Hassler J, Wang S, Pennathur S, Back SH, Katze MG, Kaufman RJ. Antioxidants Complement the Requirement for Protein Chaperone Function to Maintain ß-Cell Function and Glucose Homeostasis. Diabetes. 2015 Aug; 64(8):2892-904. PMID: 25795214.
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    40. Wang S, Park S, Kodali VK, Han J, Yip T, Chen Z, Davidson NO, Kaufman RJ. Identification of protein disulfide isomerase 1 as a key isomerase for disulfide bond formation in apolipoprotein B100. Mol Biol Cell. 2015 Feb 15; 26(4):594-604. PMID: 25518935.
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    41. Genereux JC, Qu S, Zhou M, Ryno LM, Wang S, Shoulders MD, Kaufman RJ, Lasmézas CI, Kelly JW, Wiseman RL. Unfolded protein response-induced ERdj3 secretion links ER stress to extracellular proteostasis. EMBO J. 2015 Jan 02; 34(1):4-19. PMID: 25361606.
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    42. Flaherty DP, Miller JR, Garshott DM, Hedrick M, Gosalia P, Li Y, Milewski M, Sugarman E, Vasile S, Salaniwal S, Su Y, Smith LH, Chung TD, Pinkerton AB, Aubé J, Callaghan MU, Golden JE, Fribley AM, Kaufman RJ. Discovery of Sulfonamidebenzamides as Selective Apoptotic CHOP Pathway Activators of the Unfolded Protein Response. ACS Med Chem Lett. 2014 Dec 11; 5(12):1278-1283. PMID: 25530830.
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    43. Wang M, Kaufman RJ. The impact of the endoplasmic reticulum protein-folding environment on cancer development. Nat Rev Cancer. 2014 Sep; 14(9):581-97. PMID: 25145482.
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    44. Fribley AM, Miller JR, Brownell AL, Garshott DM, Zeng Q, Reist TE, Narula N, Cai P, Xi Y, Callaghan MU, Kodali V, Kaufman RJ. Celastrol induces unfolded protein response-dependent cell death in head and neck cancer. Exp Cell Res. 2015 Jan 15; 330(2):412-22. PMID: 25139619.
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    45. Nakagawa H, Umemura A, Taniguchi K, Font-Burgada J, Dhar D, Ogata H, Zhong Z, Valasek MA, Seki E, Hidalgo J, Koike K, Kaufman RJ, Karin M. ER stress cooperates with hypernutrition to trigger TNF-dependent spontaneous HCC development. Cancer Cell. 2014 Sep 08; 26(3):331-343. PMID: 25132496; PMCID: PMC4165611.
    46. Mao HZ, Ehrhardt N, Bedoya C, Gomez JA, DeZwaan-McCabe D, Mungrue IN, Kaufman RJ, Rutkowski DT, Péterfy M. Lipase maturation factor 1 (lmf1) is induced by endoplasmic reticulum stress through activating transcription factor 6a (Atf6a) signaling. J Biol Chem. 2014 Aug 29; 289(35):24417-27. PMID: 25035425.
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    47. Bai Y, Hassler J, Ziyar A, Li P, Wright Z, Menon R, Omenn GS, Cavalcoli JD, Kaufman RJ, Sartor MA. Novel bioinformatics method for identification of genome-wide non-canonical spliced regions using RNA-Seq data. PLoS One. 2014; 9(7):e100864. PMID: 24991935.
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    48. Sadleir KR, Eimer WA, Kaufman RJ, Osten P, Vassar R. Genetic inhibition of phosphorylation of the translation initiation factor eIF2a does not block Aß-dependent elevation of BACE1 and APP levels or reduce amyloid pathology in a mouse model of Alzheimer's disease. PLoS One. 2014; 9(7):e101643. PMID: 24992504.
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    49. Di Prisco GV, Huang W, Buffington SA, Hsu CC, Bonnen PE, Placzek AN, Sidrauski C, Krnjevic K, Kaufman RJ, Walter P, Costa-Mattioli M. Translational control of mGluR-dependent long-term depression and object-place learning by eIF2a. Nat Neurosci. 2014 Aug; 17(8):1073-82. PMID: 24974795.
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    50. Cao SS, Kaufman RJ. Endoplasmic reticulum stress and oxidative stress in cell fate decision and human disease. Antioxid Redox Signal. 2014 Jul 20; 21(3):396-413. PMID: 24702237.
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    51. Yong J, Grankvist N, Han J, Kaufman RJ. Eukaryotic translation initiation factor 2 a phosphorylation as a therapeutic target in diabetes. Expert Rev Endocrinol Metab. 2014 Jul; 9(4):345-356. PMID: 30763994.
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    52. Groenendyk J, Peng Z, Dudek E, Fan X, Mizianty MJ, Dufey E, Urra H, Sepulveda D, Rojas-Rivera D, Lim Y, Kim DH, Baretta K, Srikanth S, Gwack Y, Ahnn J, Kaufman RJ, Lee SK, Hetz C, Kurgan L, Michalak M. Interplay between the oxidoreductase PDIA6 and microRNA-322 controls the response to disrupted endoplasmic reticulum calcium homeostasis. Sci Signal. 2014 Jun 10; 7(329):ra54. PMID: 24917591; PMCID: PMC4984425.
    53. Scortegagna M, Kim H, Li JL, Yao H, Brill LM, Han J, Lau E, Bowtell D, Haddad G, Kaufman RJ, Ronai ZA. Fine tuning of the UPR by the ubiquitin ligases Siah1/2. PLoS Genet. 2014 May; 10(5):e1004348. PMID: 24809345.
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    54. Cao SS, Wang M, Harrington JC, Chuang BM, Eckmann L, Kaufman RJ. Phosphorylation of eIF2a is dispensable for differentiation but required at a posttranscriptional level for paneth cell function and intestinal homeostasis in mice. Inflamm Bowel Dis. 2014 Apr; 20(4):712-22. PMID: 24577114.
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    55. Wang S, Kaufman RJ. How does protein misfolding in the endoplasmic reticulum affect lipid metabolism in the liver? Curr Opin Lipidol. 2014 Apr; 25(2):125-32. PMID: 24565920.
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    56. Kaufman RJ, Malhotra JD. Calcium trafficking integrates endoplasmic reticulum function with mitochondrial bioenergetics. Biochim Biophys Acta. 2014 Oct; 1843(10):2233-9. PMID: 24690484.
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    57. Hiramatsu N, Messah C, Han J, LaVail MM, Kaufman RJ, Lin JH. Translational and posttranslational regulation of XIAP by eIF2a and ATF4 promotes ER stress-induced cell death during the unfolded protein response. Mol Biol Cell. 2014 May; 25(9):1411-20. PMID: 24623724; PMCID: PMC4004591.
    58. Ferris SP, Kodali VK, Kaufman RJ. Glycoprotein folding and quality-control mechanisms in protein-folding diseases. Dis Model Mech. 2014 Mar; 7(3):331-41. PMID: 24609034.
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    59. Han J, Kaufman RJ. Measurement of the unfolded protein response to investigate its role in adipogenesis and obesity. Methods Enzymol. 2014; 538:135-50. PMID: 24529437.
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    60. Rajesh K, Papadakis AI, Kazimierczak U, Peidis P, Wang S, Ferbeyre G, Kaufman RJ, Koromilas AE. eIF2a phosphorylation bypasses premature senescence caused by oxidative stress and pro-oxidant antitumor therapies. Aging (Albany NY). 2013 Dec; 5(12):884-901. PMID: 24334569.
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    61. Kaufman RJ, Powell JS. Molecular approaches for improved clotting factors for hemophilia. Blood. 2013 Nov 21; 122(22):3568-74. PMID: 24065241.
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    62. Arensdorf AM, Dezwaan McCabe D, Kaufman RJ, Rutkowski DT. Temporal clustering of gene expression links the metabolic transcription factor HNF4a to the ER stress-dependent gene regulatory network. Front Genet. 2013; 4:188. PMID: 24069029.
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    63. Qiu Q, Zheng Z, Chang L, Zhao YS, Tan C, Dandekar A, Zhang Z, Lin Z, Gui M, Li X, Zhang T, Kong Q, Li H, Chen S, Chen A, Kaufman RJ, Yang WL, Lin HK, Zhang D, Perlman H, Thorp E, Zhang K, Fang D. Toll-like receptor-mediated IRE1a activation as a therapeutic target for inflammatory arthritis. EMBO J. 2013 Sep 11; 32(18):2477-90. PMID: 23942232.
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    64. Ferris SP, Jaber NS, Molinari M, Arvan P, Kaufman RJ. UDP-glucose:glycoprotein glucosyltransferase (UGGT1) promotes substrate solubility in the endoplasmic reticulum. Mol Biol Cell. 2013 Sep; 24(17):2597-608. PMID: 23864712.
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    65. Misra J, Kim DK, Choi W, Koo SH, Lee CH, Back SH, Kaufman RJ, Choi HS. Transcriptional cross talk between orphan nuclear receptor ERR? and transmembrane transcription factor ATF6a coordinates endoplasmic reticulum stress response. Nucleic Acids Res. 2013 Aug; 41(14):6960-74. PMID: 23716639.
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    66. Malhi H, Kropp EM, Clavo VF, Kobrossi CR, Han J, Mauer AS, Yong J, Kaufman RJ. C/EBP homologous protein-induced macrophage apoptosis protects mice from steatohepatitis. J Biol Chem. 2013 Jun 28; 288(26):18624-42. PMID: 23720735.
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    67. Krokowski D, Han J, Saikia M, Majumder M, Yuan CL, Guan BJ, Bevilacqua E, Bussolati O, Bröer S, Arvan P, Tchórzewski M, Snider MD, Puchowicz M, Croniger CM, Kimball SR, Pan T, Koromilas AE, Kaufman RJ, Hatzoglou M. A self-defeating anabolic program leads to ß-cell apoptosis in endoplasmic reticulum stress-induced diabetes via regulation of amino acid flux. J Biol Chem. 2013 Jun 14; 288(24):17202-13. PMID: 23645676.
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    68. Han J, Back SH, Hur J, Lin YH, Gildersleeve R, Shan J, Yuan CL, Krokowski D, Wang S, Hatzoglou M, Kilberg MS, Sartor MA, Kaufman RJ. ER-stress-induced transcriptional regulation increases protein synthesis leading to cell death. Nat Cell Biol. 2013 May; 15(5):481-90. PMID: 23624402.
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    69. Binet F, Mawambo G, Sitaras N, Tetreault N, Lapalme E, Favret S, Cerani A, Leboeuf D, Tremblay S, Rezende F, Juan AM, Stahl A, Joyal JS, Milot E, Kaufman RJ, Guimond M, Kennedy TE, Sapieha P. Neuronal ER stress impedes myeloid-cell-induced vascular regeneration through IRE1a degradation of netrin-1. Cell Metab. 2013 Mar 05; 17(3):353-71. PMID: 23473031.
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    165. Zhang B, McGee B, Yamaoka JS, Guglielmone H, Downes KA, Minoldo S, Jarchum G, Peyvandi F, de Bosch NB, Ruiz-Saez A, Chatelain B, Olpinski M, Bockenstedt P, Sperl W, Kaufman RJ, Nichols WC, Tuddenham EG, Ginsburg D. Combined deficiency of factor V and factor VIII is due to mutations in either LMAN1 or MCFD2. Blood. 2006 Mar 01; 107(5):1903-7. PMID: 16304051.
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    171. Zhang B, Kaufman RJ, Ginsburg D. LMAN1 and MCFD2 form a cargo receptor complex and interact with coagulation factor VIII in the early secretory pathway. J Biol Chem. 2005 Jul 08; 280(27):25881-6. PMID: 15886209.
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    174. Fernandez J, Yaman I, Huang C, Liu H, Lopez AB, Komar AA, Caprara MG, Merrick WC, Snider MD, Kaufman RJ, Lamers WH, Hatzoglou M. Ribosome stalling regulates IRES-mediated translation in eukaryotes, a parallel to prokaryotic attenuation. Mol Cell. 2005 Feb 04; 17(3):405-16. PMID: 15694341.
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    176. McEwen E, Kedersha N, Song B, Scheuner D, Gilks N, Han A, Chen JJ, Anderson P, Kaufman RJ. Heme-regulated inhibitor kinase-mediated phosphorylation of eukaryotic translation initiation factor 2 inhibits translation, induces stress granule formation, and mediates survival upon arsenite exposure. J Biol Chem. 2005 Apr 29; 280(17):16925-33. PMID: 15684421.
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    179. Deng J, Lu PD, Zhang Y, Scheuner D, Kaufman RJ, Sonenberg N, Harding HP, Ron D. Translational repression mediates activation of nuclear factor kappa B by phosphorylated translation initiation factor 2. Mol Cell Biol. 2004 Dec; 24(23):10161-8. PMID: 15542827.
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    180. Yamamoto K, Yoshida H, Kokame K, Kaufman RJ, Mori K. Differential contributions of ATF6 and XBP1 to the activation of endoplasmic reticulum stress-responsive cis-acting elements ERSE, UPRE and ERSE-II. J Biochem. 2004 Sep; 136(3):343-50. PMID: 15598891.
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    183. Nguyên DT, Kebache S, Fazel A, Wong HN, Jenna S, Emadali A, Lee EH, Bergeron JJ, Kaufman RJ, Larose L, Chevet E. Nck-dependent activation of extracellular signal-regulated kinase-1 and regulation of cell survival during endoplasmic reticulum stress. Mol Biol Cell. 2004 Sep; 15(9):4248-60. PMID: 15201339.
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    184. Wu S, Tan M, Hu Y, Wang JL, Scheuner D, Kaufman RJ. Ultraviolet light activates NFkappaB through translational inhibition of IkappaBalpha synthesis. J Biol Chem. 2004 Aug 13; 279(33):34898-902. PMID: 15184376.
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    185. Schröder M, Clark R, Liu CY, Kaufman RJ. The unfolded protein response represses differentiation through the RPD3-SIN3 histone deacetylase. EMBO J. 2004 Jun 02; 23(11):2281-92. PMID: 15141165.
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    187. Hsu LC, Park JM, Zhang K, Luo JL, Maeda S, Kaufman RJ, Eckmann L, Guiney DG, Karin M. The protein kinase PKR is required for macrophage apoptosis after activation of Toll-like receptor 4. Nature. 2004 Mar 18; 428(6980):341-5. PMID: 15029200.
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    189. Tardif KD, Mori K, Kaufman RJ, Siddiqui A. Hepatitis C virus suppresses the IRE1-XBP1 pathway of the unfolded protein response. J Biol Chem. 2004 Apr 23; 279(17):17158-64. PMID: 14960590.
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    190. Miao HZ, Sirachainan N, Palmer L, Kucab P, Cunningham MA, Kaufman RJ, Pipe SW. Bioengineering of coagulation factor VIII for improved secretion. Blood. 2004 May 01; 103(9):3412-9. PMID: 14726380.
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    192. Rutkowski DT, Kaufman RJ. A trip to the ER: coping with stress. Trends Cell Biol. 2004 Jan; 14(1):20-8. PMID: 14729177.
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    193. Aparna G, Bhuyan AK, Sahdev S, Hasnain SE, Kaufman RJ, Ramaiah KV. Stress-induced apoptosis in Spodoptera frugiperda (Sf9) cells: baculovirus p35 mitigates eIF2 alpha phosphorylation. Biochemistry. 2003 Dec 30; 42(51):15352-60. PMID: 14690445.
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    194. Scheuner D, Gromeier M, Davies MV, Dorner AJ, Song B, Patel RV, Wimmer EJ, McLendon RE, Kaufman RJ. The double-stranded RNA-activated protein kinase mediates viral-induced encephalitis. Virology. 2003 Dec 20; 317(2):263-74. PMID: 14698665.
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    195. Cullinan SB, Zhang D, Hannink M, Arvisais E, Kaufman RJ, Diehl JA. Nrf2 is a direct PERK substrate and effector of PERK-dependent cell survival. Mol Cell Biol. 2003 Oct; 23(20):7198-209. PMID: 14517290.
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    196. Zhang K, Kaufman RJ. Unfolding the toxicity of cholesterol. Nat Cell Biol. 2003 Sep; 5(9):769-70. PMID: 12951604.
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    197. Arnold SM, Kaufman RJ. The noncatalytic portion of human UDP-glucose: glycoprotein glucosyltransferase I confers UDP-glucose binding and transferase function to the catalytic domain. J Biol Chem. 2003 Oct 31; 278(44):43320-8. PMID: 12913004.
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    199. Jiang HY, Wek SA, McGrath BC, Scheuner D, Kaufman RJ, Cavener DR, Wek RC. Phosphorylation of the alpha subunit of eukaryotic initiation factor 2 is required for activation of NF-kappaB in response to diverse cellular stresses. Mol Cell Biol. 2003 Aug; 23(16):5651-63. PMID: 12897138.
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    200. Komiyama T, VanderLugt B, Fugère M, Day R, Kaufman RJ, Fuller RS. Optimization of protease-inhibitor interactions by randomizing adventitious contacts. Proc Natl Acad Sci U S A. 2003 Jul 08; 100(14):8205-10. PMID: 12832612.
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    201. Zhang B, Cunningham MA, Nichols WC, Bernat JA, Seligsohn U, Pipe SW, McVey JH, Schulte-Overberg U, de Bosch NB, Ruiz-Saez A, White GC, Tuddenham EG, Kaufman RJ, Ginsburg D. Bleeding due to disruption of a cargo-specific ER-to-Golgi transport complex. Nat Genet. 2003 Jun; 34(2):220-5. PMID: 12717434.
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    202. Yaman I, Fernandez J, Liu H, Caprara M, Komar AA, Koromilas AE, Zhou L, Snider MD, Scheuner D, Kaufman RJ, Hatzoglou M. The zipper model of translational control: a small upstream ORF is the switch that controls structural remodeling of an mRNA leader. Cell. 2003 May 16; 113(4):519-31. PMID: 12757712.
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    203. Knee R, Ahsan I, Mesaeli N, Kaufman RJ, Michalak M. Compromised calnexin function in calreticulin-deficient cells. Biochem Biophys Res Commun. 2003 May 16; 304(4):661-6. PMID: 12727205.
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    204. Liu CY, Kaufman RJ. The unfolded protein response. J Cell Sci. 2003 May 15; 116(Pt 10):1861-2. PMID: 12692187.
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    205. Hossain GS, van Thienen JV, Werstuck GH, Zhou J, Sood SK, Dickhout JG, de Koning AB, Tang D, Wu D, Falk E, Poddar R, Jacobsen DW, Zhang K, Kaufman RJ, Austin RC. TDAG51 is induced by homocysteine, promotes detachment-mediated programmed cell death, and contributes to the cevelopment of atherosclerosis in hyperhomocysteinemia. J Biol Chem. 2003 Aug 08; 278(32):30317-27. PMID: 12738777.
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    206. Ward SL, Scheuner D, Poppers J, Kaufman RJ, Mohr I, Leib DA. In vivo replication of an ICP34.5 second-site suppressor mutant following corneal infection correlates with in vitro regulation of eIF2 alpha phosphorylation. J Virol. 2003 Apr; 77(8):4626-34. PMID: 12663769.
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    207. Rutkowski DT, Kaufman RJ. All roads lead to ATF4. Dev Cell. 2003 Apr; 4(4):442-4. PMID: 12689582.
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    208. Reddy RK, Mao C, Baumeister P, Austin RC, Kaufman RJ, Lee AS. Endoplasmic reticulum chaperone protein GRP78 protects cells from apoptosis induced by topoisomerase inhibitors: role of ATP binding site in suppression of caspase-7 activation. J Biol Chem. 2003 Jun 06; 278(23):20915-24. PMID: 12665508.
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    209. Liu CY, Xu Z, Kaufman RJ. Structure and intermolecular interactions of the luminal dimerization domain of human IRE1alpha. J Biol Chem. 2003 May 16; 278(20):17680-7. PMID: 12637535.
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    211. Lee K, Neigeborn L, Kaufman RJ. The unfolded protein response is required for haploid tolerance in yeast. J Biol Chem. 2003 Apr 04; 278(14):11818-27. PMID: 12560331.
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    212. Kaufman RJ. Orchestrating the unfolded protein response in health and disease. J Clin Invest. 2002 Nov; 110(10):1389-98. PMID: 12438434.
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    213. Hakeos WH, Miao H, Sirachainan N, Kemball-Cook G, Saenko EL, Kaufman RJ, Pipe SW. Hemophilia A mutations within the factor VIII A2-A3 subunit interface destabilize factor VIIIa and cause one-stage/two-stage activity discrepancy. Thromb Haemost. 2002 Nov; 88(5):781-7. PMID: 12428094.
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    214. Deng J, Harding HP, Raught B, Gingras AC, Berlanga JJ, Scheuner D, Kaufman RJ, Ron D, Sonenberg N. Activation of GCN2 in UV-irradiated cells inhibits translation. Curr Biol. 2002 Aug 06; 12(15):1279-86. PMID: 12176355.
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    218. Lee K, Tirasophon W, Shen X, Michalak M, Prywes R, Okada T, Yoshida H, Mori K, Kaufman RJ. IRE1-mediated unconventional mRNA splicing and S2P-mediated ATF6 cleavage merge to regulate XBP1 in signaling the unfolded protein response. Genes Dev. 2002 Feb 15; 16(4):452-66. PMID: 11850408.
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    219. Tallóczy Z, Jiang W, Virgin HW, Leib DA, Scheuner D, Kaufman RJ, Eskelinen EL, Levine B. Regulation of starvation- and virus-induced autophagy by the eIF2alpha kinase signaling pathway. Proc Natl Acad Sci U S A. 2002 Jan 08; 99(1):190-5. PMID: 11756670.
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