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2020

Amplifiers co-translationally enhance CFTR biosynthesis via PCBP1-mediated regulation of CFTR mRNA

Dukovski, D., Villella, A., Bastos, C., King, R., Finley, D., Kelly, J. W., Morimoto, R. I., Hartl, F. U., Munoz, B., Lee, P. S., Zecevic, M. & Miller, J. P., Jan 1 2020, (Accepted/In press) In : Journal of Cystic Fibrosis.

Research output: Contribution to journalArticle

Open Access
1 Scopus citations

Cell-nonautonomous regulation of proteostasis in aging and disease

Morimoto, R. I., Apr 2020, In : Cold Spring Harbor Perspectives in Biology. 12, 4, a034074.

Research output: Contribution to journalArticle

Open Access
3 Scopus citations
2019

A metastable subproteome underlies inclusion formation in muscle proteinopathies

Ciryam, P., Antalek, M., Cid, F., Tartaglia, G. G., Dobson, C. M., Guettsches, A. K., Eggers, B., Vorgerd, M., Marcus, K., Kley, R. A., Morimoto, R. I., Vendruscolo, M. & Weihl, C. C., Dec 3 2019, In : Acta Neuropathologica Communications. 7, 1, 197.

Research output: Contribution to journalArticle

Open Access

Deconvoluting Stress-Responsive Proteostasis Signaling Pathways for Pharmacologic Activation Using Targeted RNA Sequencing

Grandjean, J. M. D., Plate, L., Morimoto, R. I., Bollong, M. J., Powers, E. T. & Luke Wiseman, R., Apr 19 2019, In : ACS chemical biology. 14, 4, p. 784-795 12 p.

Research output: Contribution to journalArticle

2 Scopus citations

Tau protein aggregates inhibit the protein-folding and vesicular trafficking arms of the cellular proteostasis network

Yu, A., Fox, S. G., Cavallini, A., Kerridge, C., O'Neill, M. J., Wolak, J., Bose, S. & Morimoto, R. I., May 10 2019, In : Journal of Biological Chemistry. 294, 19, p. 7917-7930 14 p.

Research output: Contribution to journalArticle

3 Scopus citations
2018

Large-scale investigation of the reasons why potentially important genes are ignored

Stoeger, T., Gerlach, M., Morimoto, R. I. & Nunes Amaral, L. A., Sep 2018, In : PLoS biology. 16, 9, e2006643.

Research output: Contribution to journalArticle

39 Scopus citations

Metformin Targets Mitochondrial Electron Transport to Reduce Air-Pollution-Induced Thrombosis

Soberanes, S., Misharin, A. V., Jairaman, A., Morales-Nebreda, L., McQuattie-Pimentel, A. C., Cho, T., Hamanaka, R. B., Meliton, A. Y., Reyfman, P. A., Walter, J. M., Chen, C-I., Chi, M., Chiu, S., Gonzalez-Gonzalez, F. J., Antalek, M., Abdala-Valencia, H., Chiarella, S. E., Sun, K. A., Woods, P. S., Ghio, A. J. & 12 others, Jain, M., Perlman, H., Ridge, K. M., Morimoto, R. I., Sznajder, J. I., Balch, W. E., Bhorade, S. M., Bharat, A., Prakriya, M., Chandel, N. S., Mutlu, G. M. & Budinger, G. R. S., Oct 11 2018, In : Cell Metabolism.

Research output: Contribution to journalArticle

9 Scopus citations

Reply to “Far away from the lamppost”

Stoeger, T., Gerlach, M., Morimoto, R. I. & Amaral, L. A. N., Dec 2018, In : PLoS biology. 16, 12, e3000075.

Research output: Contribution to journalArticle

2017

In vivo properties of the disaggregase function of J-proteins and Hsc70 in Caenorhabditis elegans stress and aging

Kirstein, J., Arnsburg, K., Scior, A., Szlachcic, A., Guilbride, D. L., Morimoto, R. I., Bukau, B. & Nillegoda, N. B., Dec 2017, In : Aging Cell. 16, 6, p. 1414-1424 11 p.

Research output: Contribution to journalArticle

15 Scopus citations

Long-range correlations and fractal dynamics in C. elegans: Changes with aging and stress

Alves, L. G. A., Winter, P. B., Ferreira, L. N., Brielmann, R. M., Morimoto, R. I. & Amaral, L. A. N., Aug 29 2017, In : Physical Review E. 96, 2, 022417.

Research output: Contribution to journalArticle

6 Scopus citations

Mitochondrial Stress Restores the Heat Shock Response and Prevents Proteostasis Collapse during Aging

Labbadia, J., Brielmann, R. M., Neto, M. F., Lin, Y. F., Haynes, C. M. & Morimoto, R. I., Nov 7 2017, In : Cell reports. 21, 6, p. 1481-1494 14 p.

Research output: Contribution to journalArticle

32 Scopus citations

Monocyte-derived alveolar macrophages drive lung fibrosis and persist in the lung over the life span

Misharin, A. V., Morales-Nebreda, L., Reyfman, P. A., Cuda, C. M., Walter, J. M., McQuattie-Pimentel, A. C., Chen, C. I., Anekalla, K. R., Joshi, N., Williams, K. J. N., Abdala-Valencia, H., Yacoub, T. J., Chi, M., Chiu, S., Gonzalez-Gonzalez, F. J., Gates, K., Lam, A. P., Nicholson, T. T., Homan, P. J., Soberanes, S. & 23 others, Dominguez, S., Morgan, V. K., Saber, R., Shaffer, A., Hinchcliff, M., Marshall, S. A., Bharat, A., Berdnikovs, S., Bhorade, S. M., Bartom, E. T., Morimoto, R. I., Balch, W. E., Sznajder, J. I., Chandel, N. S., Mutlu, G. M., Jain, M., Gottardi, C. J., Singer, B. D., Ridge, K. M., Bagheri, N., Shilatifard, A., Budinger, G. R. S. & Perlman, H., Aug 1 2017, In : Journal of Experimental Medicine. 214, 8, p. 2387-2404 18 p.

Research output: Contribution to journalArticle

173 Scopus citations

Protein homeostasis of a metastable subproteome associated with Alzheimer's disease

Kundra, R., Ciryam, P., Morimoto, R. I., Dobson, C. M. & Vendruscolo, M., Jul 11 2017, In : Proceedings of the National Academy of Sciences of the United States of America. 114, 28, p. E5703-E5711

Research output: Contribution to journalArticle

25 Scopus citations

Spinal motor neuron protein supersaturation patterns are associated with inclusion body formation in ALS

Ciryam, P., Lambert-Smith, I. A., Bean, D. M., Freer, R., Cid, F., Tartaglia, G. G., Saunders, D. N., Wilson, M. R., Oliver, S. G., Morimoto, R. I., Dobson, C. M., Vendruscolo, M., Favrin, G. & Yerbury, J. J., May 16 2017, In : Proceedings of the National Academy of Sciences of the United States of America. 114, 20, p. E3935-E3943

Research output: Contribution to journalArticle

34 Scopus citations

The Intersection of Aging Biology and the Pathobiology of Lung Diseases: A Joint NHLBI/NIA Workshop

Budinger, G. S., Kohanski, R. A., Gan, W., Kobor, M. S., Amaral, L. A., Armanios, M., Kelsey, K. T., Pardo, A., Tuder, R., Macian, F., Chandel, N., Vaughan, D., Rojas, M., Mora, A. L., Kovacs, E., Duncan, S. R., Finkel, T., Choi, A., Eickelberg, O., Chen, D. & 8 others, Agusti, A., Selman, M., Balch, W. E., Busse, P., Lin, A., Morimoto, R., Sznajder, J. I. & Thannickal, V. J., Nov 1 2017, In : Journals of Gerontology - Series A Biological Sciences and Medical Sciences. 72, 11, p. 1492-1500 9 p.

Research output: Contribution to journalArticle

19 Scopus citations
2016

A network approach to discerning the identities of C. elegans in a free moving population

Winter, P. B., Brielmann, R. M., Timkovich, N. P., Navarro, H. T., Teixeira-Castro, A., Morimoto, R. I. & Amaral, L. A. N., Oct 11 2016, In : Scientific Reports. 6, 34859.

Research output: Contribution to journalArticle

3 Scopus citations

A transcriptional signature of Alzheimer's disease is associated with a metastable subproteome at risk for aggregation

Ciryam, P., Kundra, R., Freer, R., Morimoto, R. I., Dobson, C. M. & Vendruscolo, M., Apr 26 2016, In : Proceedings of the National Academy of Sciences of the United States of America. 113, 17, p. 4753-4758 6 p.

Research output: Contribution to journalArticle

40 Scopus citations

E2F coregulates an essential HSF developmental program that is distinct from the heat-shock response

Li, J., Chauve, L., Phelps, G., Brielmann, R. M. & Morimoto, R. I., Sep 15 2016, In : Genes and Development. 30, 18, p. 2062-2075 14 p.

Research output: Contribution to journalArticle

30 Scopus citations

Susan Lindquist 1949-2016

Morimoto, R. I., Dec 6 2016, In : Nature structural & molecular biology. 23, 12, p. 1072-1073 2 p.

Research output: Contribution to journalArticle

1 Scopus citations
2015

Crucial HSP70 co-chaperone complex unlocks metazoan protein disaggregation

Nillegoda, N. B., Kirstein, J., Szlachcic, A., Berynskyy, M., Stank, A., Stengel, F., Arnsburg, K., Gao, X., Scior, A., Aebersold, R., Guilbride, D. L., Wade, R. C., Morimoto, R. I., Mayer, M. P. & Bukau, B., Aug 13 2015, In : Nature. 524, 7564, p. 247-251 5 p.

Research output: Contribution to journalArticle

140 Scopus citations

Investigating the spreading and toxicity of prion-like proteins using the metazoan model organism C. Elegans

Nussbaum-Krammer, C. I., Neto, M. F., Brielmann, R. M., Pedersen, J. S. & Morimoto, R. I., Jan 8 2015, In : Journal of Visualized Experiments. 95, e52321.

Research output: Contribution to journalArticle

55 Scopus citations

Neuronal serotonin release triggers the heat shock response in C elegans in the absence of temperature increase

Tatum, M. C., Ooi, F. K., Chikka, M. R., Chauve, L., Martinez-Velazquez, L. A., Steinbusch, H. W. M., Morimoto, R. I. & Prahlad, V., Jan 19 2015, In : Current Biology. 25, 2, p. 163-174 12 p.

Research output: Contribution to journalArticle

56 Scopus citations

Proteotoxic stress and ageing triggers the loss of redox homeostasis across cellular compartments

Kirstein, J., Morito, D., Kakihana, T., Sugihara, M., Minnen, A., Hipp, M. S., Nussbaum-Krammer, C., Kasturi, P., Hartl, F. U., Nagata, K. & Morimoto, R. I., Sep 1 2015, In : EMBO Journal. 34, 18, p. 2334-2349 16 p.

Research output: Contribution to journalArticle

44 Scopus citations

Repression of the Heat Shock Response Is a Programmed Event at the Onset of Reproduction

Labbadia, J. & Morimoto, R. I., Aug 20 2015, In : Molecular Cell. 59, 4, p. 639-650 12 p.

Research output: Contribution to journalArticle

103 Scopus citations

Serotonergic signalling suppresses ataxin 3 aggregation and neurotoxicity in animal models of Machado-Joseph disease

Teixeira-Castro, A., Jalles, A., Esteves, S., Kang, S., Da Silva Santos, L., Silva-Fernandes, A., Neto, M. F., Brielmann, R. M., Bessa, C., Duarte-Silva, S., Miranda, A., Oliveira, S., Neves-Carvalho, A., Bessa, J., Summavielle, T., Silverman, R. B., Oliveira, P., Morimoto, R. I. & Maciel, P., Nov 2015, In : Brain. 138, 11, p. 3221-3237 17 p.

Research output: Contribution to journalArticle

35 Scopus citations

Tertiary Amine Pyrazolones and Their Salts as Inhibitors of Mutant Superoxide Dismutase 1-Dependent Protein Aggregation for the Treatment of Amyotrophic Lateral Sclerosis

Zhang, Y., Zhao, K. T., Fox, S. G., Kim, J., Kirsch, D. R., Ferrante, R. J., Morimoto, R. I. & Silverman, R. B., Jul 17 2015, In : Journal of Medicinal Chemistry. 58, 15, p. 5942-5949 8 p.

Research output: Contribution to journalArticle

5 Scopus citations

Widespread proteome remodeling and aggregation in aging C. elegans

Walther, D. M., Kasturi, P., Zheng, M., Pinkert, S., Vecchi, G., Ciryam, P., Morimoto, R. I., Dobson, C. M., Vendruscolo, M., Mann, M. & Hartl, F. U., May 7 2015, In : Cell. 161, 4, p. 919-932 14 p.

Research output: Contribution to journalArticle

222 Scopus citations
2014

A chaperome subnetwork safeguards proteostasis in aging and neurodegenerative disease

Brehme, M., Voisine, C., Rolland, T., Wachi, S., Soper, J. H., Zhu, Y., Orton, K., Villella, A., Garza, D., Vidal, M., Ge, H. & Morimoto, R. I., Jan 1 2014, In : Cell reports. 9, 3, p. 1135-1150 16 p.

Research output: Contribution to journalArticle

193 Scopus citations

Characterizing the altered cellular proteome induced by the stress-independent activation of heat shock factor 1

Ryno, L. M., Genereux, J. C., Naito, T., Morimoto, R. I., Powers, E. T., Shoulders, M. D. & Wiseman, R. L., Jun 20 2014, In : ACS chemical biology. 9, 6, p. 1273-1283 11 p.

Research output: Contribution to journalArticle

21 Scopus citations

Deuteration and fluorination of 1,3-bis(2-phenylethyl)pyrimidine-2,4,6(1H,3H,5H)-trione to improve its pharmacokinetic properties

Xia, G., Benmohamed, R., Morimoto, R. I., Kirsch, D. R. & Silverman, R. B., Nov 1 2014, In : Bioorganic and Medicinal Chemistry Letters. 24, 21, p. 5098-5101 4 p.

Research output: Contribution to journalArticle

4 Scopus citations

Dysregulation of the proteasome increases the toxicity of ALS-linked mutant SOD1

Kitamura, A., Inada, N., Kubota, H., Matsumoto, G., Kinjo, M., Morimoto, R. I. & Nagata, K., Mar 1 2014, In : Genes to Cells. 19, 3, p. 209-224 16 p.

Research output: Contribution to journalArticle

26 Scopus citations

Malfolded protein structure and proteostasis in lung diseases

Balch, W. E., Sznajder, J. I., Budinger, GR. S., Finley, D., Laposky, A. D., Cuervo, A. M., Benjamin, I. J., Barreiro, E., Morimoto, R. I., Postow, L., Weissman, A. M., Gail, D., Banks-Schlegel, S., Croxton, T. & Gan, W., Jan 1 2014, In : American journal of respiratory and critical care medicine. 189, 1, p. 96-103 8 p.

Research output: Contribution to journalArticle

31 Scopus citations

Modulation of the Maladaptive Stress Response to Manage Diseases of Protein Folding

Roth, D. M., Hutt, D. M., Tong, J., Bouchecareilh, M., Wang, N., Seeley, T., Dekkers, J. F., Beekman, J. M., Garza, D., Drew, L., Masliah, E., Morimoto, R. I. & Balch, W. E., Jan 1 2014, In : PLoS biology. 12, 11

Research output: Contribution to journalArticle

53 Scopus citations

Proteasome activation is a mechanism for pyrazolone small molecules displaying therapeutic potential in amyotrophic lateral sclerosis

Trippier, P. C., Zhao, K. T., Fox, S. G., Schiefer, I. T., Benmohamed, R., Moran, J., Kirsch, D. R., Morimoto, R. I. & Silverman, R. B., Sep 17 2014, In : ACS Chemical Neuroscience. 5, 9, p. 823-829 7 p.

Research output: Contribution to journalArticle

24 Scopus citations

Protein aggregation can inhibit clathrin-mediated endocytosis by chaperone competition

Yu, A., Shibata, Y., Shah, B., Calamini, B., Lo, D. C. & Morimoto, R. I., Apr 15 2014, In : Proceedings of the National Academy of Sciences of the United States of America. 111, 15, p. E1481-E1490

Research output: Contribution to journalArticle

72 Scopus citations
2013

Arylazanylpyrazolone derivatives as inhibitors of mutant superoxide dismutase 1 dependent protein aggregation for the treatment of amyotrophic lateral sclerosis

Zhang, Y., Benmohamed, R., Huang, H., Chen, T., Voisine, C., Morimoto, R. I., Kirsch, D. R. & Silverman, R. B., Mar 28 2013, In : Journal of Medicinal Chemistry. 56, 6, p. 2665-2675 11 p.

Research output: Contribution to journalArticle

11 Scopus citations

Heat shock response activation exacerbates inclusion body formation in a cellular model of huntington disease

Bersuker, K., Hipp, M. S., Calamini, B., Morimoto, R. I. & Kopito, R. R., Aug 16 2013, In : Journal of Biological Chemistry. 288, 33, p. 23633-23638 6 p.

Research output: Contribution to journalArticle

34 Scopus citations

Identification of a Tissue-Selective Heat Shock Response Regulatory Network

Guisbert, E., Czyz, D. M., Richter, K., McMullen, P. D. & Morimoto, R. I., Apr 1 2013, In : PLoS genetics. 9, 4, e1003466.

Research output: Contribution to journalArticle

57 Scopus citations

In vivo translation rates can substantially delay the cotranslational folding of the Escherichia coli cytosolic proteome

Ciryam, P., Morimoto, R. I., Vendruscolo, M., Dobson, C. M. & O'Brien, E. P., Jan 8 2013, In : Proceedings of the National Academy of Sciences of the United States of America. 110, 2, p. E132-E140

Research output: Contribution to journalArticle

50 Scopus citations

Natural genetic variation determines susceptibility to aggregation or toxicity in a C. elegans model for polyglutamine disease

Gidalevitz, T., Wang, N., Deravaj, T., Alexander-Floyd, J. & Morimoto, R. I., Sep 30 2013, In : BMC Biology. 11, 100.

Research output: Contribution to journalArticle

22 Scopus citations

Neuronal Reprograming of Protein Homeostasis by Calcium-Dependent Regulation of the Heat Shock Response

Silva, M. C., Amaral, M. D. & Morimoto, R. I., Aug 1 2013, In : PLoS Genetics. 9, 8, e1003711.

Research output: Contribution to journalArticle

12 Scopus citations

Regulation of organismal proteostasis by transcellular chaperone signaling

Van Oosten-Hawle, P., Porter, R. S. & Morimoto, R. I., Jun 6 2013, In : Cell. 153, 6, 1 p.

Research output: Contribution to journalArticle

103 Scopus citations

Spreading of a Prion Domain from Cell-to-Cell by Vesicular Transport in Caenorhabditis elegans

Nussbaum-Krammer, C. I., Park, K. W., Li, L., Melki, R. & Morimoto, R. I., Apr 15 2013, In : PLoS genetics. 9, 3, e1003351.

Research output: Contribution to journalArticle

32 Scopus citations

The nascent polypeptide-associated complex is a key regulator of proteostasis

Kirstein-Miles, J., Scior, A., Deuerling, E. & Morimoto, R. I., May 15 2013, In : EMBO Journal. 32, 10, p. 1451-1468 18 p.

Research output: Contribution to journalArticle

85 Scopus citations

Widespread Aggregation and Neurodegenerative Diseases Are Associated with Supersaturated Proteins

Ciryam, P., Tartaglia, G. G., Morimoto, R. I., Dobson, C. M. & Vendruscolo, M., Nov 7 2013, In : Cell reports. 5, 3, p. 781-790 10 p.

Research output: Contribution to journalArticle

121 Scopus citations
2012
31 Scopus citations
46 Scopus citations

Chiral cyclohexane 1,3-diones as inhibitors of mutant SOD1-dependent protein aggregation for the treatment of ALS

Zhang, Y., Benmohamed, R., Zhang, W., Kim, J., Edgerly, C. K., Zhu, Y., Morimoto, R. I., Ferrante, R. J., Kirsch, D. R. & Silverman, R. B., Jul 12 2012, In : ACS Medicinal Chemistry Letters. 3, 7, p. 584-587 4 p.

Research output: Contribution to journalArticle

9 Scopus citations

Cyclohexane 1,3-diones and their inhibition of mutant SOD1-dependent protein aggregation and toxicity in PC12 cells

Zhang, W., Benmohamed, R., Arvanites, A. C., Morimoto, R. I., Ferrante, R. J., Kirsch, D. R. & Silverman, R. B., Jan 15 2012, In : Bioorganic and Medicinal Chemistry. 20, 2, p. 1029-1045 17 p.

Research output: Contribution to journalArticle

14 Scopus citations

Dynamic imaging by fluorescence correlation spectroscopy identifies diverse populations of polyglutamine oligomers formed in vivo

Beam, M., Silva, M. C. & Morimoto, R. I., Jul 27 2012, In : Journal of Biological Chemistry. 287, 31, p. 26136-26145 10 p.

Research output: Contribution to journalArticle

15 Scopus citations