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Chao J. T., Pina, F., and Niwa, M. (2020) The Regulations of Early Stages of Endoplasmic Reticulum Inheritance in ER stress. Mol. Biol. Cell. (Accepted for Publication) 

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Niwa, M. (2020) A Cell Cycle Checkpoint for the Endoplasmic Reticulum. BBA Molecular Cell Research 1867 118825-

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Das, B., Okamoto, K., Rabalais, J., Marchelletta, R. R., Barrett, K. E., Das, S., Niwa, M., and Sivagnanam, M (2020) Congenital Tufting Enterophathy-Associated Mutant of Epithelial Cell Adhesion Molecule Activates the Unfolded Protein Response in a Murine Model of the Disease. Cells 9: 946.


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*Chao, J. T., Pina, F., Onishi, M., Cohen, Y., Lai, Y., Schuldiner, M., and Niwa, M. (2019) Transfer of the septin ring to cytokinetic Remnants in ER stress directs age-sensitive cell cycle re-entry. Developmental Cell 51: 173-191.

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Tam A. B., Lindsay, S. R., Chandra, V., Nomura, K. D., Forbes, D. J., and Niwa, M. (2018) The UPR Activator ATF6 Responds to Proteotoxic and Lipotoxic Stress by Distinct Mechanisms. Developmental Cell 46:327-343.

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*Pina-Nunez, F. N. Yagisawa, F., Obara, K., Gregerson, J. D., Kihara, A., and Niwa M. (2018) Sphingolipids activate the Endoplasmic Reticulum Stress Surveillance (ERSU) pathway.

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J of Cell Bio. 217 (2) 495-505


Miller, M., Tam, A. B., Mueller, J., L., Rosenthal, P., Beppu, A., Gordillo, R., McGeough, M. D., Voung, C., Doherty, T. A., Hoffman, H. M., Suzukawa, M., Niwa, M, and Broide, D. H. (2017) Targeting epithelial ORMDL3 increases, rather than reduces, airway responsiveness through increased sphingosine-1-phosphate. J of Immnol. 198:3017-3022.


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*Pina-Nunez, F. N., Fleming, T., Pogliano, K., and Niwa, M. (2016) Reticulons are important for ER inheritance regulation and function during ER stress. Developmental Cell. 37: 279-288.

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Chao, J. T., and Niwa, M. (2016) Nursing the Follicles. Developmental Cell 37: 7-8.

Jiang D, Lynch C, Medeiros BC, Liedtke M, Bam R, Tam AB, Yang Z, Alagappan M, Abidi P, Le QT, Giaccia AJ, Denko NC, Niwa M, Koong AC. (2016) Identification of Doxorubicin as an inhibitor of the IRE1a-XBP1 Axis of the Unfolded Protein Response. Sci Rep. 6: 33353.


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DOI:10.1038/srep33353 or

Jiang, D., Niwa, M., and Koong, A. C.(2016) Targeting the IRE1a-XBP1 Branch of Unfolded Protein Response in Human Diseases. Seminars in Cancer Biology, 33: 45-56. (PMCID:PMC4523453)

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Jiang D, Tam AB, Alagappan M, Hay MP, Gupta A, Kozak MM, Solow-Cordero DE, Lum PY, Denko NC, Giaccia AJ, Le QT, Niwa M, Koong AC. (2016) Acridine Derivatives as Inhibitors of the IRE1α-XBP1 Pathway Are Cytotoxic to Human Multiple Myeloma. Mol Cancer Ther. 15: 2055-65. (PMCID:PMC5010920)

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DOI: 10.1158/1535-7163.MCT-15-1023

*Pina-Nunez, F. N., Niwa, M. (2015) Initiation of the Endoplasmic Reticulum (ER) inheritance is determined by the ER functional status during the cell cycle. eLife 4:e06970

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Tam, A. B., Koong, A. C., and Niwa, M. (2014) IRE1 Differentially Cleaves HAC1/XBP1 RNA and ER-Associated RNA. Cell Reports 9:850-858

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Miller, M., Rosenthal, P., Beppu, A., Mueller, J. L., Hoffman, H. M. Tam, A. B., Doherty, T. A., McGeough, M. D., Pena, C., Suzukawa, M., Niwa, M., and Broide, D. H. (2014) ORMDL3 transgenic mice have increased airway remodeling characteristic of asthma. J. immunology. 192: 3475-87

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Miller, M., Tam, A. B., Cho, J. Y., Doherty, T. A., Pham, A., Khorram, N., Rosenthal, P., Mueller, J. L., Hoffman, H. M., Suzukawa, M., Niwa, M, and Broide, D. H. (2012) ORMDL3 is an inducible lung epithelial gene regulating metalloproteases, chemokines, OAS, and ATF6. Proc Natl Acad Sci U S A. 109:16648-53

(PMID:23011799 PMCID/PMC3478632)

Tam, A. B., O’Dea, E., Hoffmann, A., and Niwa M. (2012) ER stress activates NF-kB by integrating function of basal IKK activity, IRE1 and PERK. PLoS One 7(10):e45078

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(PMID:23110043, PMCID:PMC3482226)

Martin-Perez, R., Niwa, M., and Lopez-Rivas, A. (2012) ER stress sensitizes cells to TRAIL through down-regulation of FLIP and Mcl-1 and PERK-dependent up-regulation of TRAIL-R2. Apoptosis 17: 349-63.

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(PMID:22072062,  PMCID:PMC4096301)

Chawla, A., Chakrabarti, S., Ghosh, G., and Niwa M. (2011) Attenuation of the yeast UPR response is essential for survival and is mediated by IRE1 kinase. J. Cell Biol.  193:41-50.

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Papandreou, I., Olson, M., Van Melckebeke, H., Lust, S., Tam, A., Solow-Cordero, D. E., Bouley, D. M., Denko, N., Offner, F., Niwa, M. & Koong, A. C. (2011) Identification of a small-molecule inhibitor of Ire1a endonuclease activity with cytotoxic activity against multiple myeloma. Blood 117: 1311-1314.

(PMID:21081713,  PMCID:PMC3056474)

*Babour A., Bicknell, A. B. Tourtellotte, J., and Niwa M. (2010) An organelle surveillance pathway monitors functional fitness of the endoplasmic reticulum to control its inheritance. Cell 142: 256-269. (PMC3359143)

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Bicknell A., Tourtellotte J, and Niwa M. (2010) Late Phase of the Endoplasmic Reticulum Stress Response Pathway is Regulated by Hog1 MAP Kinase. J of Biol. Chem. 285: 17545-17555. (PMC2878519)

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Bicknell A., and Niwa M. (2009) Regulating ER function through the Unfolded Protein Response. The Handbook of Cell Signaling 2nd ed., Oxford: Academic Press. 2511-2525.


DuRose, J., Kaufman, R. A., Rothblum, L., and Niwa M. (2009) PERK regulates both cellular translation repression and RRN3 mediated rRNA transcription down-regulation during the Unfolded Protein Response pathway. Mol Cell Biol. 29: 4295-4307.

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DOI: 10.1128/MCB.00260-09

Brunsing, R., Oomori, S., Weber, F., Ware, A., Friend, L., Rickert, R., and Niwa, M. (2008) B- and T-cell development both involve activity of the Unfolded Protein Response pathway. J. Biol. Chem. 283: 17954-17961.


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https://doi: 10.1074/jbc.M801395200

Bicknell A.A., Babour A., Federovitch C.M., and Niwa M. (2007) A Novel role for the Unfolded Protein Response (UPR) in cytokinesis reveals previously uncharacterized housekeeping function for the UPR. J. Cell Bio.l 177: 1017-1027.

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DuRose, J., Tam A., and Niwa M. (2006) Molecular mechanism of differential activation of three ER proximal UPR components. Mol. Biol. Cell 17, 3095-3107


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Chawla, A., and Niwa M. (2005) The Unfolded Protein Response signaling pathway. Current Biology 15, R907.

Niwa M. (2005) Unfolded Protein Response Pathway. (2005) Topics in Current Genetics 10, 35-63.

Niwa M., Patel C., DeRisi J., and Walter P. (2005) Non-conventional HAC1 mRNA splicing provides a private communication pathway between the endoplasmic reticulum and the nucleus. Genome Biology 6:R3.1-3.10.  


Nock S., Gonzalez T., Niwa M., and Walter P. (2001) Purification and activity assays of the catalytic domains of the kinase/nuclease Ire1p from S. cerevisiae. Methods in Enzymology 342:3-10

Niwa M., and Walter P. (2000) Pausing to decide. Proc. Nat. Acad. Sci. 97, 12396-12397.

(PMID:11058174,  PMCID:PMC34056)

Dong B., Niwa M., Walter P., and Silverman R. H. (2000). Basis for regulated RNA cleavage by functional analysis of RNaseL and Ire1p. RNA 7, 361-373.

(PMID:11333017   PMCID:PMC1370093)


Niwa M., Sidrauski, C., and Walter P. (1999). A Role of Presenilin-1 in nuclear accumulation of Ire1 fragments and induction of the mammalian Unfolded Protein Response. Cell 99, 1-20.


Brown J. D., Hann B. C., Medzihradszky K. F., Niwa M., Burlingame A. L., and Walter P. Subunits of the Saccharomyces cerevisiae signal recognition particle required for its functional expression. (1994). EMBO J. 13, 4390-4400.

PMID:7925282,  PMCID:PMC395366

Lowell C. A, Niwa M., Soriano P., and Varmus H. E. (1996). Deficiency of the Hck and Src tyrosine kinases results in extreme levels of extramedullary hematopoiesis. Blood 87, 1780-92.


Niwa, M., MacDonald, C. C., and Berget, S. M. (1992). Are vertebrate exons scanned during splice site selection.  Nature 360, 277-280.



Niwa, M., and Berget, S. M. (1991). Polyadenylation precedes splicing in vitro. Gene Expression 1, 5-14.

(PMID:1726467    PMCID:PMC5952195)

Niwa, M., and Berget, S. M. (1991). Mutation of the AAUAAA polyadenylation signal depresses in vitro splicing of proximal but not distal introns. Genes Dev. 5, 2086-2095.


doi: 10.1101/gad.5.11.2086,

Niwa, M., Rose, S.D., and Berget, S. M. (1990). In vitro polyadenylation is stimulated by the presence of an upstream intron. Genes Dev. 4, 1552-1559.