Publications


2014

Unanue ER, UranoF. Endoplasmic Reticulum: an interface between the immune system and metabolism. Diabetes. 2014: 63(1):48-9.

Hara T, Mahadevan J, Kanekura K, Hara M, Lu S, Urano F. Calcium efflux from the endoplasmic reticulum leads to β cell death. Endocrinology. 2014. 155(3):758-6.

Urano F. Targeting endoplasmic reticulum to combat juvenile diabetes. Nature Reviews Endocrinology. 2014. 0(3):129-30.

Urano F. Wolfram syndrome iPS Cells: The First Human Cell Model of Endoplasmic Reticulum Disease. Diabetes. 2014. 63(3):844-6.

Liew CW, Assmann A, Templin AT, Raum JC, Lipson KL, Rajan S, Qiang G, Hu J, Kawamori D, Lindberg I, Philipson LH, Sonenberg N, Goldfine AB, Stoffers DA, Mirmira RG, Urano F, Kulkarni RN. Proc Natl Acad Sci USA. 2014. In press.


2013

Tohmonda T, Yoda M, Mizuochi H, Morioka H, Matsumoto M, Urano F, Toyama Y, Horiuchi K. The IRE1α-XBP1 pathway positively regulates parathyroid hormone (PTH)/PTH-related peptide receptor expression and is involved in pth-induced osteoclastogenesis. J Biol Chem. 2013: 288(3):1691-5. PMCID: PMC3548479

Sama RR, Ward CL, Kaushansky LJ, Lemay N, Ishigaki S, Urano F, Bosco DA. FUS/TLS assembles into stress granules and is a prosurvival factor during hyperosmolar stress. J Cell Physiol. 2013: 228(11): 2222-31.

Marshall BA, Permutt MA, Paciorkowski AR, Hoekel J, Karzon R, Wasson J, Viehover A, White NH, Shimony JS, Manwaring L, Austin P, Hullar TE, Hershey T; Washington University Wolfram Study Group. Phenotypic characteristics of early Wolfram syndrome. J Rare Dis. 2013: 8(1):64.

Bonnycastle LL, Chines PS, Hara T, Huyghe JR, Swift AJ, Heikinheimo P, Mahadevan J, Peltonen S, Huopio H, Nuutila P, Narisu N, Goldfeder RL, Stitzel ML, Lu S, Boehnke M, Urano F, Collins FS, Laakso M. Autosomal Dominant Diabetes Arising from a Wolfram Syndrome 1. Diabetes. 2013: 62(11):3943-50.

Kanekura K, Ishigaki S, Merksamer PI, Papa FR, Urano F. Establishment of a system for monitoring endoplasmic reticulum redox state in mammalian cells. Lab Invest. 2013. 93(11): 1254-8

Yang C, diIorio P, Jurczyk A, O’Sullivan-Murphy B, Urano F, Bortell R. Pathological endoplasmic reticulum stress mediated by the IRE1 pathway contributes to pre-insulitic beta cell apoptosis in a virus-induced rat model of type 1 diabetes. Diabetologia. 2013: 56(12):2638-46.

Jung DY, Chalasani U, Pan N, Friedline RH, Prosdocimo DA, Nam M, Azuma Y, Maganti R, Yu K, Velagapudi A, O’Sullivan-Murphy B, Sartoretto JL, Jain MK, Cooper MP, Urano F, Kim JK, Gray S.KLF15 is a molecular link between endoplasmic reticulum stress and insulin resistance. PLoS One. 2013: 8(10):e77851.

Jurczyk A, diIorio P, Brostowin D, Leehy L, Yang C, Urano F, Harlan DM, Shultz LD, Greiner DL, Bortell R. Improved function and proliferation of adult human beta cells engrafted in diabetic immunodeficient NOD-scid IL2rgnull mice treated with alogliptin. Diabetes Metab Syndr Obes. 2013. 13(6):493-9.


2012

Sharma M, Urano F, Jaeschke A. Cdc42 and Rac1 are major contributors to the saturated fatty acid-stimulated JNK pathway in hepatocytes. J Hepatol 2012: 56(1): 192-198. PMCID: PMC3183327

Matsuoka K, Hayashi S, Urano F, Zhu LJ, Okita H, Sago H, Nakazawa A. Squamous metaplasia in the cyst epithelium of type 1 congenital pulmonary airway malformation after thoracoamniotic shunt placement. Human Path 2012: 43(9):1413-7.

Alexandrov I, Ivshina M, Jung DY, Friedline R, Ko HJ, Xu M, O’Sullivan-Murphy B, Bortell R, Urano F, Kim JK, Richter J. Cytoplasmic polyadenylation binding protein deficiency stimulates PTEN and Stat3 mRNA translation and induces hepatic insulin resistance. PLoS Genetics 2012: 8(1): e1002457. PMCID: PMC3257279

O’Sullivan-Murphy B, Urano F. ER stress as a trigger for beta cell dysfunction and autoimmunity in type 1 diabetes. Diabetes 2012: 61(4): 780-781. PMCID: PMC3314361

Kondo S, Hino S, Saito A, Kanemoto S, Kawasaki N, Asada R, Izumi S, Iwamoto H, Oki M, Miyagi H, Kaneko M, Nomura Y, Urano F, Imaizumi K.: Activation of OASIS family, ER stress transducers, is dependent on its stabilization. Cell Death Diff, 2012: 19(12): 1939-49. PMCID: PMC3504707

Oslowski CM, Hara T, O’Sullivan-Murphy B, Kanekura K, Lu S, Hara M, Ishigaki S, Hayashi E, Hui ST, Greiner D, Kaufman RJ, Bortell R, Urano F. TXNIP mediates ER stress-induced beta cell death through the initiation of the inflammasome. Cell Metab 2012: 16(2): 265-7. PMCID: PMC3418541

Ishigaki S, Masuda A, Fujioka Y, Iguchi Y, Katsuno M, Shibata A, Urano F, Sobue G, Ohno K. Position-dependent FUS-RNA interactions regulate alternative splicing events and transcriptions. Sci Rep. 2012: 2:529. PMCID: PMC3402842

Fonseca SG, Urano F, Weir GC, Gromada J, Burcin M. WFS1 and adenylyl cyclase 8 interact at the plasma membrane to regulate insulin production and secretion. Nature Cell Bio 2012: 14(10): 1105-12. PMCID: PMC3589109

Katsumata R, Ishigaki S, Katsuno M, Kawai K, Sone J, Huang Z, Adachi H, Tanaka F, Urano F, Sobue G. c-Abl Inhibition Delays Motor Neuron Degeneration in the G93A Mouse, an Animal Model of Amyotrophic Lateral Sclerosis. PLoS One 2012: 7(9):e46185. PMCID: PMC3458026

Pickett KA, Duncan RP, Hoekel J, Marshall B, Hershey T, Earhart GM; Washington University Wolfram Study Group. Early presentation of gait impairment in Wolfram Syndrome. Orphanet J Rare Dis. 2012: 7:92. PMCID: PMC3551701

O’Sullivan-Murphy B, Urano F. Pathological ER stress in cells. 2012. In: Agostinis P, Afshin S, Eds. Endoplasmic Reticulum Stress in Health and Disease. Pages 215-230. Springer.

Kanekura K, Lu S, Lipson KL, Urano F. Role of ER Stress in Dysfunction of the Nervous System. 2012. In: Agostinis P, Afshin S, Eds. Endoplasmic Reticulum Stress in Health and Disease. Pages 299-321. Springer.

Urano F, Matsuoka K. Pulmonary Pathology. 2012. In. Nakazawa A, Matsuoka K, Okita H, Eds. Color Atlas of Pediatric Pathology. Chapter 3. Diagnostics and Therapeutics, Tokyo, Japan.


2011

Oslowski, C.M. and Urano, F. The binary switch that controls the life and death decisions of ER stressed beta cells. Curr Opin Cell Biol, 23(2): 207-15, 2011.

Oslowski, C.M. and Urano, F. Measuring ER stress and the unfolded protein response using mammalian tissue culture system. Methods in Enzymology, 490: 71-92, 2011.

Tohmonda, T., Miyauchi, Y., Ghosh, R., Yoda, M., Uchikawa, S., Takito, J., Morioka, H., Nakamura, M., Iwawaki, T., Chiba, K., Toyama, Y., Urano, F. and Horiuchi, K. The IRE1–XBP1 pathway is essential for osteoblast differentiation through promoting transcription of Osterix. EMBO Reports, 12(5):451-7, 2011.

Fonseca, S.G., Gromada, J., and Urano, F. Endoplasmic reticulum stress and pancreatic cell death. Trends in Endcrinol and Metab, 22(7): 266-74, 2011.

Meares, G., Hughes, K., Naatz, A., Papa, F., Urano, F., Hansen, P., Benveniste, E., and Corbett, J. IRE1-dependent activation of AMPK in response to nitric oxide, Mol Cell Biol, 31(21): 4286-97, 2011.


2010

Ishigaki, S., Fonseca, S.G., Oslowski, C.M, Jurczyk, A., Shearstone, J.R., Zhu, L.J., Permutt, M.A., Greiner, D.L., Bortell, R., and Urano, F. AATF mediates anti-apoptotic effect of the unfolded protein response through transcriptional regulation of AKT1. Cell Death Differ, 17(5):774-86, 2010

Fonseca, S.G., Urano, F., Burcin, M., and Gromada, J. Stress HypERactivation in the Beta Cell. Islets, 2(1): 1-9, 2010.

Oslowski, C.M., and Urano, F. The binary switch between life and death of ER stressed beta cells. Curr Opin Endocrinol, Diabetes and Obes, 17(2):107-12, 2010.

Fonseca, S.G., Ishigaki, S., Oslowski, C.M, Lu, S., Lipson, K.L., Ghosh, R., Hayashi, E., Ishihara, H., Oka, Y., Permutt, M.A., and Urano, F. Wolfram syndrome 1 gene negatively regulates ER stress signaling in rodent and human cells. J Clin Invest, 120(3):744-55, 2010.

Ghosh R, Lipson KL, Sargent KE, Mercurio AM, Hunt JS, Ron D, Urano F. Transcriptional Regulation of VEGF-A by the Unfolded Protein Response Pathway. PLoS One, 5(3): e9575, 2010.

Oslowski, C.M., and Urano, F. A Switch From Life To Death in ER stressed beta cells. Diabetes Obes Metab, Suppl 2:58-65, 2010.

Jurczyk, A., Pino, S., O’Sullivan-Murphy, B., Addorio, M., Lidstone, E., DiIorio, P., Lipson, K., Standley, C., Fogarty, K., Lifshitz, L., Urano, F., Mordes, J., Greiner, D., Rossini, A. and Bortell R. Novel Role for the Centrosomal Protein, Pericentrin, in Regulation of Insulin Secretory Vesicle Docking in Mouse Pancreatic Beta-cells. PLoS One, 5(7):e11812, 2010.


2009

Kakiuchi, C., Ishigaki, S., Oslowski, C.M., Fonseca, S.G., Kato, T., and Urano, F. Valproate, a mood stabilizer, induces WFS1 expression and modulates its interaction with ER stress protein GRP94. PLoS One, 4(1):e4134, 2009.

Fonseca, S.G., Burcin, M., Gromada, J., and Urano, F. Endoplasmic Reticulum Stress in Beta Cells and Development of Diabetes. Curr Opin Pharmacol, 9(6): 763-70, 2009.


2008

Chambers, K.T., Unverferth, J.A., Weber, S.M., Wek, R.C., Urano, F., and Corbett, J.A. The Role of Nitric Oxide and the Unfolded Protein Response in Cytokine Induced beta-cell Death. Diabetes, 57(1): 124-132, 2008.

Lipson, K.L., Ghosh, R., and Urano, F. The role of IRE1α in the degradation of insulin mRNA in pancreatic β-cells. PLoS One, 3(2): e1648, 2008.

Luo, D., Yu, L., Zhang, H., He, Y., Urano, F., and Min, W. AIP1 is critical in transducing IRE1-mediated ER stress response. J Biol Chem, 283(18):11905-12, 2008.

Pino, S.C., O’Sullivan-Murphy, B., Lidstone, E.A., Thornley, T.B., Jurczyk, A., Urano, F., Greiner, D.L., Mordes, J.P., Rossini, A.A, and Bortell, R. Protein Kinase C Signaling during T Cell Activation Induces the Endoplasmic Reticulum Stress Response. Cell Stress & Chaperone, 13(4): 421-34, 2008.


2007

Ishigaki, S., Niwa, J., Yamada, S., Takahashi, M., Ito, T., Sone, J., Doyu, M., Urano, F., and Sobue, G.Dorfin-CHIP chimeric proteins potently ubiquitylate and degrade familial ALS-related mutant SOD1 proteins and reduce their cellular toxicity. Neurobiology of Disease, 25(2): 231-41, 2007.

Fonseca, S.G., Lipson, K.L, and Urano, F. Endoplasmic reticulum stress signaling in pancreatic beta cells. Antioxid Redox Signal, 9(12): 2335-44, 2007.


2006

Lipson, K., Fonseca, S.G., and Urano, F. Endoplasmic reticulum stress and diabetes (Review article). Current Mol Medicine, 6(1): 71-77, 2006.

Lipson, K., Fonseca, S.G, Nguyen, L., Foss, E., Bortell, R., Rossini, A., and Urano, F. Regulation of insulin biosynthesis in pancreatic beta-cells by an endoplasmic reticulum-resident protein kinase IRE1. Cell Metabolism, 4(3), 245-254, 2006.

Ogata, M., Saito, A., Morikawa, K., Kondo, S., Kanemoto, S., Murakami, T., Taniguchi, M., Tanii, I, Yoshinaga, K., Hammarback, J.A., Urano, F., and Imaizumi, K. Autophagy is activated during ER stress mediated by an ER-resident transmembrane protein kinase IRE1. Mol Cell Biol, 26(24): 9220-31, 2006


2005

Fonseca, S.G.., Fukuma, M., Lipson, K., Nguyen, L.X., Allen, J.R., Oka, Y., and Urano F. WFS1 is a novel component of the unfolded protein response and maintains ER homeostasis in pancreatic beta cells. J Biol Chem, 280(47): 39609-39615, 2005.

Kubota, K., Lee, D.H., Everett, E.T., Martinez-Mier, E.A., Snead, M.L., Nguyen, L., Urano, F., and Bartlett, J.D. Fluoride induces ER stress in ameloblasts responsible for dental enamel formation. J Biol Chem, 280(24): 23194-202, 2005.

Kadowaki, H., Nishitoh, H., Urano, F., Sadamitsu, C., Matsuzawa, A., Takeda, K., Masutani, H., Yodoi, J., Urano, Y., Nagano, T. and Ichijo, H. Amyloid beta induces neuronal cell death through ROS-mediated ASK1 activation. Cell Death Differ, 12(1): 19-24, 2005.

Kanemoto, S., Kondo, S., Ogata, M., Murakami, T., Urano, F., and Imaizumi, K. XBP1 activates the transcription of its target gene via an ACGT core sequence under ER stress. Biochem Biophys Res Commun, 331: 1146-1153, 2005.


2004

Allen, J.R., Nguyen, L.X., Sargent, K.E.G., Lipson, K.L., Hackett, A., and Urano, F. High ER stress in beta cells stimulates intracellular degradation of misfolded insulin. Biochem Biophys Res Commun, 324: 166-170, 2004.

Yoneda, T., Benedetti, C., Urano, F., Clark, S.G., Harding, H.P., and Ron, D. Compartment-specific perturbation of protein handling activates genes encoding mitochondrial chaperone. J Cell Sci, 117: 4055-4066, 2004.


2003

Matsushita, K., Okita, H., Suzuki, A., Shimoda, K., Fukuma, M., Yamada, T., Urano, F., Honda, T., Sano, M., Iwanaga, S., Ogawa, S., Hata, J., and Umezawa, A. Islet cell hyperplasia in transgenic mice overexpressing EAT/mcl-1, a bcl-2 related gene. Mol Cell Endcrinol, 203: 105-116, 2003.


2002

Calfon, M., Zeng, H., Urano, F., Till, JH., Hubbard, SR., Harding, HP., Clark, SG., and Ron D. IRE1 couples endoplasmic reiculum load to secretory capacity by processing the XBP-1 mRNA. Nature, 415: 92-96, 2002.

Yabe, H., Fukuma, M., Urano, F., Yoshida, K., Kato, S.,Toyama, Y., Hata, J-i., and Umezawa, A. Lack of Matrix Metalloproteinase (MMP)-1 and -3 expression in Ewing’s sarcoma may be due to loss of accessibility of the MMP regulatory element to the specific fusion protein in vivo. Biochem Biophys Res Commun, 293: 61-71, 2002.

Yoshizawa, J., Maie, M., Eto, T., Higashimoto, Y., Saito, T., Horie, H., and Urano, F. A case of intra-abdominal desmoplastic small-round-cell tumor with elevated serum CA125. Pediatr Surg Int, 18: 238-240, 2002.

Yoneda, T., Urano, F., and Ron, D. Transmission of proteotoxicity across cellular compartments. Genes and Dev, 16: 1307-1313, 2002.

Urano, F., Calfon, M., Yoneda, T., Yun, C., Kiraly, M., Clark, SG., and Ron, D. A survival pathway for C.elegans with a blocked unfolded protein response. J Cell Biol, 158: 639-646, 2002.

Harding, H., Calfon, M., Urano, F., Novoa, I. and Ron, D. Transcriptional and translational control in the mammalian unfolded protein response. Annual Rev Cell Dev Biol, 18: 575-599, 2002.


2001

Jousse, C., Bruhat, A., Carraro, V., Urano, F., Ferrara, M., Ron, D., and Fafournoux, P. Inhibition of CHOP translation by peptide encoded by an open reading frame localized in the chop 5’ UTR. Nucleic Acid Res, 29(21):4341-51, 2001.

Harding, H., Novoa, I, Bertolotti, A., Zeng, Hl, Zhang, Y., Urano, F., Jousse, C., and Ron, D. Translational regulation in the cellular response to biosynthetic load on the endoplasmic reticulum. Cold Spring Harb Symp Quant Biol, LXVI: 499-508, 2001.


2000

Okita, H., Umezawa, A., Fukuma, M., Ando, T., Urano, F., Sano, M., Nakata, Y., Mori, T., and Hata, J-i. Acute myeloid leukemia possessing jumping translocation is related to highly elevated levels of EAT/mcl-1, a Bcl-2 related gene with anti-apoptotic functions. Leukemia Res, 24(1): 73-77, 2000.

Urano, F. , Wang, X-Z., Bertolloti, A., Zhang, Y., Chung, P., Harding, H. and Ron, D. Coupling of stress in the ER to activation of JNK protein kinases by transmembrane protein kinase IRE1. Science, 287: 664-666, 2000.

Kuroda, M., Sok, J., Webb, L., Baechtold, H., Urano, F., Yin, Y., Chung, P., de Rooij DG., Akhmedov, A., Ashley, T., and Ron, D. Male sterility and enhanced radiation sensitivity in TLS(-/-) mice. EMBO J, 19(3): 453-462, 2000.

Sato, N., Urano, F., Leem, JY., Kim, SH., Li, M., Donoviel, D., Bernstein, A., Lee, AS., Ron, D., Veselits, ML., Sisodia, SS., and Thinakaran, G. Upregulation of BiP and CHOP by the unfolded-protein response is independent of Presenilin expression. Nature Cell Biol, 2: 863-879, 2000.

Urano, F., Bertolotti, A., and Ron, D. IRE1 and efferent signaling from endoplasmic reticulum. J Cell Science, 113: 3697-3702, 2000.


1999

Fukuzawa, R., Umezawa, A., Ochi, K., Urano, F., Ikeda, H., and Hata, J-i. High frequency of inactivation of the imprinted H19 gene in “sporadic” hepatoblastoma. Int J Cancer, 82(4): 490-497, 1999.


1998

Urano, F., Umezawa, A., Yabe, H., Hong, W., Yoshida, K., Fujinaga, K., and Hata, J-i. Molecular analysis of Ewing’s sarcoma: another fusion gene, EWS-E1AF, available for the diagnosis. Jpn J Cancer Res, 89: 703-711, 1998.

Ishida, S., Yoshida, K., Kaneko, Y., Tanaka, Y., Sasaki, Y., Urano, F., Umezawa, A., Hata, J, and Fujinaga, K. The genomic breakpoint and chimeric transcripts in the EWSR1-ETV4/E1AF gene fusion in Ewing sarcoma. Cytogenet and Cell Genet, 82: 278-283, 1998.


1996

Urano, F., Umezawa, A., Hong, W., Kikuchi, H., and Hata, J-i. A novel chimera gene between EWS and E1A-F, encoding the adenovirus E1A enhancer-binding protein, in extraosseous Ewing’s sarcoma. Biochem Biophys Res Commun, 219: 608-612, 1996