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Lynne Elizabeth Maquat, Ph.D.

Lynne Elizabeth Maquat, Ph.D.

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Connect with Lynne Elizabeth Maquat, Ph.D.

About Me

Professional Background

Dr. Lynne Maquat is the J. Lowell Orbison Endowed Chair and Professor of Biochemistry & Biophysics who holds concomitant appointments in Pediatrics and in Oncology, Founding Director of the Center for RNA Biology, and Founding Chair of Graduate Women in Science at the University of Rochester, Roches...
Dr. Lynne Maquat is the J. Lowell Orbison Endowed Chair and Professor of Biochemistry & Biophysics who holds concomitant appointments in Pediatrics and in Oncology, Founding Director of the Center for RNA Biology, and Founding Chair of Graduate Women in Science at the University of Rochester, Rochester, NY. After obtaining her PhD in Biochemistry from the University of Wisconsin-Madison and undertaking post-doctoral work at the McArdle Laboratory for Cancer Research, she joined Roswell Park Cancer Institute before moving to the University of Rochester Medical Center. Dr. Maquat’s research focuses on the molecular basis of human diseases, with particular interest in mechanisms of mRNA decay. Dr. Maquat discovered nonsense-mediated mRNA decay (NMD) in human diseases in 1981 and, subsequently, the exon-junction complex (EJC) and how the EJC marks mRNAs for a quality-control “pioneer” round of protein synthesis. She also discovered Staufen-mediated mRNA decay, which mechanistically competes with NMD and, by so doing, new roles for short interspersed elements and long non-coding RNAs. Additionally, she has defined a new mechanism by which microRNAs are degraded, thereby regulating mRNAs so as to promote the cell cycle. One of her current interests focuses on the development of therapeutics for diseases that she has shown manifest hyperactivated NMD, including the most common single gene cause of intellectual disability and autism, Fragile X Syndrome. Maquat is an elected Fellow of the American Association for the Advancement of Science (2006); an elected Member of the American Academy of Arts & Sciences (2006), the National Academy of Sciences (2011), and the National Academy of Medicine (2017); and a Batsheva de Rothschild Fellow of the Israel Academy of Sciences & Humanities (2012-3). She received the William C. Rose Award from the American Society for Biochemistry & Molecular Biology (2014), a Canada Gairdner International Award (2015), the international RNA Society Lifetime Achievement Award in Service (2010) and in Science (2017), the FASEB Excellence in Science Award (2018), the Vanderbilt Prize in Biomedical Science (2017), the Wiley Prize in Biomedical Sciences from Rockefeller University (2018), the International Union of Biochemistry and Molecular Biology Medal (2019), the Wolf Prize in Medicine from Isreal (2021), the Warren Alpert Foundation Prize from Harvard Medical School (2021), and the Gruber Genetics Prize from the Gruber Foundation and Yale University (2023). Maquat is well-known for her efforts to promote women in science.

Faculty Appointments

Professor - Department of Biochemistry and Biophysics (SMD)

Director, Center for RNA Biology - Office of VP for Health Sciences (URMC)

J. Lowell Orbison Distinguished Service Alumni Professorship - Dean's Office, SMD (SMD)

Credentials

Education

PhD | Univ Wisconsin-Madison. Biochemistry. 1979

BA | University of Connecticut. Biology. 1974

Awards

Gruber Genetics Prize. 2023

Wolf Prize in Medicine. 2021

Warren Alpert Prize. 2021

Warren Alpert Foundation Prize. 2021

IUBMB Jubilee Award. 2019

FASEB Excellence in Science Award. 2018

Wiley Prize in Biomedical Sciences. 2018

2017 International RNA Society Lifetime Achievement in Science Award. 2017

2017 Vanderbilt Prize in Biomedical Science. 2017

Elected to the National Academy of Medicine. 2017

Canada Gairdner International Award. 2015

William C. Rose Award. 2014

Athena Award. 2014

Presidential Diversity Award. 2013

Batsheva de Rothschild Fellow of the Israel Academy of Sciences and Humanities. 2013

MERIT Award from the NIH GM. 2013

Elected to the National Academy of Sciences. 2011

RNA Society Lifetime Achievement Award in Service. 2010

Elected to the American Association for the Advancement of Science. 2006

Elected to the American Academy of Arts and Sciences. 2006

Davey Memorial Award for Outstanding Cancer Research. 2002

RPI/RNA Award for most significant paper co-authored by a junior scientist published in RNA. 1998

Exemplary "Woman in Government". 1990

American Heart Association Established Investigatorship. 1985 - 1989

Research

Research in my lab focuses on RNA decay pathways. One pathway, called nonsense-mediated mRNA decay (NMD) or mRNA surveillance, surveys all newly synthesized mRNAs during what we call a "pioneer" round of translation. This round of translation involves mRNA that is associated with the cap-binding het...
Research in my lab focuses on RNA decay pathways. One pathway, called nonsense-mediated mRNA decay (NMD) or mRNA surveillance, surveys all newly synthesized mRNAs during what we call a "pioneer" round of translation. This round of translation involves mRNA that is associated with the cap-binding heterodimer CBP80 and CBP20. It is distinct from the type of translation that supports the bulk of cellular protein synthesis and involves a different cap-binding protein, eukaryotic initiation factor (eIF) 4E. Generally, if translation terminates more than 50-55 nt upstream of an exon-exon junction that is marked by the NMD factors Upf3 or Upf3X, Upf2 and ultimately Upf1, then the mRNA will be subject to NMD. By the time CBP80 and CBP20 have been replaced by eIF4E, the Upf mark has been removed so that mRNA is largely immune to NMD.

Studies in progress will significantly advance our understanding of the mRNP proteins, translation factors and nucleases that trigger NMD. Our results will be useful when designing therapies that aim to abrogate NMD in order to abrogate the severity of nonsense-generated diseases. We are also interested in further characterizing the pioneer translation initiation complex and requirements for its remodeling to the steady-state initiation complex that involves eIF4E. Additionally, we are interested in the cycle of posttranslational modifications that typify at least some of the NMD factors, including phosphorylation of Upf1 that is mediated by the PI 3-kinase-related protein kinase Smg1.

Over the past 15 years, our discovery and subsequent work on the mechanism of Staufen (Stau)-mediated mRNA decay (SMD) has uncovered new roles for cytoplasmic long non-coding RNAs (lncRNAs) and retrotransposon-derived short interspersed elements (SINEs) in post-transcriptional gene regulation. These SINEs include human Alu elements and mouse B1, B2, B4 and ID elements. We have shown that NMD and SMD are competitive pathways in ways that contribute to cellular homeostasis and also differentiation. We continue to define new cellular roles for SINEs as sites for nucleating intermolecular base-pairing between different mRNAs, between mRNAs and lncRNAs, and between different lncRNAs. We are additionally extending our studies of inverted-repeat Alu elements (IRAlus) and how competitive binding among the many nuclear and cytoplasmic double-stranded RNA binding proteins influence nuclear and cytoplasmic IRAlus-containing RNA metabolism.

Most recently, we have discovered a new microRNA decay pathway that is mediated by Tudor-SN. This pathway, which we call TumiD, promotes G1-to-S phase transition by degrading microRNAs that degrade mRNAs encoding proteins that promote this transition. We are currently working on how TumiD is regulated.

Please visit our lab website for more information on our exciting past and ongoing research pursuits as well as opportunities available to join our group.

Patents

Methods and Compositions Related to Staufen 1 Binding Sites Formed By Duplexing ALU Elements

Issue date: December 08, 2015

Patent #: 9,206,479

Country: United States

Inventors: Chenguang Gong, Lynne Elizabeth Maquat

Publications

Journal Articles

ALS-Associated TDP-43 Dysfunction Compromises UPF1-Dependent mRNA Metabolism Pathways Including Alternative Polyadenylation and 3'UTR Length.

Alessandrini F, Wright M, Kurosaki T, Maquat LE, Kiskinis E

bioRxiv : the preprint server for biology.. 2024 February 2 Epub 02/02/2024.

FMRP-mediated spatial regulation of physiologic NMD targets in neuronal cells.

Kurosaki T, Rambout X, Maquat LE

Genome biology.. 2024 January 2325 (1):31. Epub 01/23/2024.

PGC-1? senses the CBC of pre-mRNA to dictate the fate of promoter-proximally paused RNAPII.

Rambout X, Cho H, Blanc R, Lyu Q, Miano JM, Chakkalakal JV, Nelson GM, Yalamanchili HK, Adelman K, Maquat LE

Molecular cell.. 2023 January 1983 (2):186-202.e11. Epub 1900 01 01.

Integrative omics indicate FMRP sequesters mRNA from translation and deadenylation in human neuronal cells.

Kurosaki T, Mitsutomi S, Hewko A, Akimitsu N, Maquat LE

Molecular cell.. 2022 November 1 Epub 11/01/2022.

AKT constitutes a signal-promoted alternative exon-junction complex that regulates nonsense-mediated mRNA decay.

Cho H, Abshire ET, Popp MW, Pröschel C, Schwartz JL, Yeo GW, Maquat LE

Molecular cell.. 2022 June 3 Epub 06/03/2022.

Lessons from the functional characterization of lncRNAs: introduction to mammalian genome special issue.

Maquat L, Johnson R, Valadkhan S, Carninci P

Mammalian genome : official journal of the International Mammalian Genome Society.. 2022 May 9 Epub 05/09/2022.

Noncoding RNAs: biology and applications-a Keystone Symposia report.

Cable J, Heard E, Hirose T, Prasanth KV, Chen LL, Henninger JE, Quinodoz SA, Spector DL, Diermeier SD, Porman AM, Kumar D, Feinberg MW, Shen X, Unfried JP, Johnson R, Chen CK, Wilusz JE, Lempradl A, McGeary SE, Wahba L, Pyle AM, Hargrove AE, Simon MD, Marcia M, Przanowska RK, Chang HY, Jaffrey SR, Contreras LM, Chen Q, Shi J, Mendell JT, He L, Song E, Rinn JL, Lalwani MK, Kalem MC, Chuong EB, Maquat LE, Liu X

Annals of the New York Academy of Sciences.. 2021 November 17 Epub 11/17/2021.

NMD abnormalities during brain development in the Fmr1-knockout mouse model of fragile X syndrome.

Kurosaki T, Sakano H, Pröschel C, Wheeler J, Hewko A, Maquat LE

Genome biology.. 2021 November 1622 (1):317. Epub 11/16/2021.

Loss of the fragile X syndrome protein FMRP results in misregulation of nonsense-mediated mRNA decay.

Kurosaki T, Imamachi N, Pröschel C, Mitsutomi S, Nagao R, Akimitsu N, Maquat LE

Nature cell biology.. 2021 January 23 (1):40-48. Epub 01/08/2021.

NCBP3: A Multifaceted Adaptive Regulator of Gene Expression.

Rambout X, Maquat LE

Trends in biochemical sciences.. 2020 October 5 Epub 10/05/2020.

The nuclear cap-binding complex as choreographer of gene transcription and pre-mRNA processing.

Rambout X, Maquat LE

Genes & development.. 2020 September 134 (17-18):1113-1127. Epub 1900 01 01.

Viral subversion of nonsense-mediated mRNA decay.

Popp MW, Cho H, Maquat LE

RNA.. 2020 July 22 Epub 07/22/2020.

Transcriptional Coactivator PGC-1? Binding to Newly Synthesized RNA via CBP80: A Nexus for Co- and Posttranscriptional Gene Regulation.

Rambout X, Cho H, Maquat LE

Cold Spring Harbor symposia on quantitative biology.. 2020 April 15 Epub 04/15/2020.

Short interspersed nuclear element (SINE)-mediated post-transcriptional effects on human and mouse gene expression: SINE-UP for active duty.

Maquat LE

Philosophical transactions of the Royal Society of London. Series B, Biological sciences.. 2020 March 30375 (1795):20190344. Epub 02/10/2020.

Cellular RNA surveillance in health and disease.

Wolin SL, Maquat LE

Science.. 2019 November 15366 (6467):822-827. Epub 11/14/2019.

Publisher Correction: Quality and quantity control of gene expression by nonsense-mediated mRNA decay.

Kurosaki T, Popp MW, Maquat LE

Nature reviews. Molecular cell biology.. 2019 April 26 Epub 04/26/2019.

Quality and quantity control of gene expression by nonsense-mediated mRNA decay.

Kurosaki T, Popp MW, Maquat LE

Nature reviews. Molecular cell biology.. 2019 April 16 Epub 04/16/2019.

UPFront and center in RNA decay: UPF1 in nonsense-mediated mRNA decay and beyond.

Kim YK, Maquat LE

RNA.. 2019 January 17 Epub 01/17/2019.

Evaluating the susceptibility of AGO2-loaded microRNAs to degradation by nucleases in vitro.

Elbarbary, R.A. and Maquat, L.E.

Methods: MicroRNA biogenesis, identification, function and decay. 2019; 152: 18-22.

Defining nonsense-mediated mRNA decay intermediates in human cells.

Kurosaki, T., Myers, J. and Maquat, L.E.

Methods: Biochemical and next generation sequencing approaches to study RNA regulation. 2019; .

Defining nonsense-mediated mRNA decay intermediates in human cells.

Kurosaki T, Myers JR, Maquat LE

Methods : a companion to Methods in enzymology.. 2018 December 19 Epub 12/19/2018.

Publisher Correction: NMD-degradome sequencing reveals ribosome-bound intermediates with 3'-end non-templated nucleotides.

Kurosaki T, Miyoshi K, Myers JR, Maquat LE

Nature structural & molecular biology.. 2018 November 25 (11):1059. Epub 1900 01 01.

NMD-degradome sequencing reveals ribosome-bound intermediates with 3'-end non-templated nucleotides.

Kurosaki T, Miyoshi K, Myers JR, Maquat LE

Nature structural & molecular biology.. 2018 October 25 (10):940-950. Epub 10/01/2018.

Evaluating the susceptibility of AGO2-loaded microRNAs to degradation by nucleases in vitro.

Elbarbary R, Maquat LE

Methods : a companion to Methods in enzymology.. 2018 May 17 Epub 05/17/2018.

Beyond Transcription: Roles of Transcription Factors in Pre-mRNA Splicing.

Rambout X, Dequiedt F, Maquat LE

Chemical reviews.. 2018 April 25118 (8):4339-4364. Epub 12/18/2017.

Introduction-RNA: From Single Molecules to Medicine.

Walter NG, Maquat LE

Chemical reviews.. 2018 April 25118 (8):4117-4119. Epub 1900 01 01.

Transcriptional coactivator PGC-1? contains a novel CBP80-binding motif that orchestrates efficient target gene expression.

Cho H, Rambout X, Gleghorn ML, Nguyen PQT, Phipps CR, Miyoshi K, Myers JR, Kataoka N, Fasan R, Maquat LE

Genes & development.. 2018 April 132 (7-8):555-567. Epub 04/13/2018.

Molecular autopsy provides evidence for widespread ribosome-phased mRNA fragmentation.

Kurosaki T, Maquat LE

Nature structural & molecular biology.. 2018 April 25 (4):299-301. Epub 1900 01 01.

Nonsense-mediated mRNA Decay and Cancer.

Popp MW, Maquat LE

Current opinion in genetics & development.. 2018 February 48 :44-50. Epub 11/07/2017.

Evidence for convergent evolution of SINE-directed Staufen-mediated mRNA decay.

Lucas BA, Lavi E, Shiue L, Cho H, Katzman S, Miyoshi K, Siomi MC, Carmel L, Ares M, Maquat LE

Proceedings of the National Academy of Sciences of the United States of America.. 2018 January 30115 (5):968-973. Epub 01/16/2018.

UPF1 helicase promotes TSN-mediated miRNA decay.

Elbarbary RA, Miyoshi K, Hedaya O, Myers JR, Maquat LE

Genes & development.. 2017 July 1531 (14):1483-1493. Epub 08/21/2017.

Distinct mechanisms obviate the potentially toxic effects of inverted-repeat Alu elements on cellular RNA metabolism.

Elbarbary RA, Maquat LE

Nature structural & molecular biology.. 2017 June 624 (6):496-498. Epub 1900 01 01.

Tudor-SN-mediated endonucleolytic decay of human cell microRNAs promotes G/S phase transition.

Elbarbary RA, Miyoshi K, Myers JR, Du P, Ashton JM, Tian B, Maquat LE

Science.. 2017 May 26356 (6340):859-862. Epub 1900 01 01.

Crystal structure of a poly(rA) staggered zipper at acidic pH: evidence that adenine N1 protonation mediates parallel double helix formation.

Gleghorn ML, Zhao J, Turner DH, Maquat LE

Nucleic acids research.. 2016 September 3044 (17):8417-24. Epub 06/10/2016.

Leveraging Rules of Nonsense-Mediated mRNA Decay for Genome Engineering and Personalized Medicine.

Popp MW, Maquat LE

Cell.. 2016 June 2165 (6):1319-1322. Epub 1900 01 01.

Eukaryotic antisense ahead of its time.

Maquat LE

Nature reviews. Molecular cell biology.. 2016 April 17 (4):204. Epub 02/24/2016.

Coupling pre-mRNA splicing and 3' end formation to mRNA export: alternative ways to punch the nuclear export clock.

Elbarbary RA, Maquat LE

Genes & development.. 2016 March 130 (5):487-8. Epub 1900 01 01.

Retrotransposons as regulators of gene expression.

Elbarbary RA, Lucas BA, Maquat LE

Science.. 2016 February 12351 (6274):aac7247. Epub 02/11/2016.

Nonsense-mediated mRNA decay in humans at a glance.

Kurosaki T, Maquat LE

Journal of cell science.. 2016 February 1129 (3):461-7. Epub 01/19/2016.

CARMing down the SINEs of anarchy: two paths to freedom from paraspeckle detention.

Elbarbary RA, Maquat LE

Genes & development.. 2015 April 129 (7):687-9. Epub 1900 01 01.

Attenuation of nonsense-mediated mRNA decay facilitates the response to chemotherapeutics.

Popp MW, Maquat LE

Nature communications.. 2015 March 266 :6632. Epub 03/26/2015.

RNA. A TRICK'n way to see the pioneer round of translation.

Popp MW, Maquat LE

Science.. 2015 March 20347 (6228):1316-7. Epub 1900 01 01.

Preservation of forelimb function by UPF1 gene therapy in a rat model of TDP-43-induced motor paralysis.

Jackson KL, Dayton RD, Orchard EA, Ju S, Ringe D, Petsko GA, Maquat LE, Klein RL

Gene therapy.. 2015 January 22 (1):20-8. Epub 11/06/2014.

Affinity purification of long noncoding RNA-protein complexes from formaldehyde cross-linked mammalian cells.

Gong C, Maquat LE

Methods in molecular biology.. 2015 1206 :81-6. Epub 1900 01 01.

A post-translational regulatory switch on UPF1 controls targeted mRNA degradation.

Kurosaki T, Li W, Hoque M, Popp MW, Ermolenko DN, Tian B, Maquat LE

Genes & development.. 2014 September 128 (17):1900-16. Epub 1900 01 01.

'Black sheep' that don't leave the double-stranded RNA-binding domain fold.

Gleghorn ML, Maquat LE

Trends in biochemical sciences.. 2014 July 39 (7):328-40. Epub 06/19/2014.

Defective secretory-protein mRNAs take the RAPP.

Popp MW, Maquat LE

Trends in biochemical sciences.. 2014 April 39 (4):154-6. Epub 03/03/2014.

The dharma of nonsense-mediated mRNA decay in mammalian cells.

Popp MW, Maquat LE

Molecules and cells.. 2014 January 37 (1):1-8. Epub 01/27/2014.

Dodging two bullets with one dsRNA-binding protein.

Elbarbary RA, Maquat LE

Cell cycle.. 2014 13 (3):345-6. Epub 12/13/2013.

Mobile DNA: an evolving field.

Belfort M, Marraffini L, Macfarlan T, Boeke J, Slotkin K, Malik H, Maquat L

Mobile DNA. 2014 5 :16. Epub 06/05/2014.

mRNA-mRNA duplexes that autoelicit Staufen1-mediated mRNA decay.

Gong C, Tang Y, Maquat LE

Nature structural & molecular biology.. 2013 October 20 (10):1214-20. Epub 09/22/2013.

STAU1 binding 3' UTR IRAlus complements nuclear retention to protect cells from PKR-mediated translational shutdown.

Elbarbary RA, Li W, Tian B, Maquat LE

Genes & development.. 2013 July 127 (13):1495-510. Epub 1900 01 01.

New gene expression pipelines gush lncRNAs.

Wang J, Lucas BA, Maquat LE

Genome biology.. 2013 May 2414 (5):117. Epub 05/24/2013.

Control of myogenesis by rodent SINE-containing lncRNAs.

Wang J, Gong C, Maquat LE

Genes & development.. 2013 April 127 (7):793-804. Epub 04/04/2013.

Staufen1 dimerizes through a conserved motif and a degenerate dsRNA-binding domain to promote mRNA decay.

Gleghorn ML, Gong C, Kielkopf CL, Maquat LE

Nature structural & molecular biology.. 2013 April 20 (4):515-24. Epub 03/24/2013.

Temporal and spatial characterization of nonsense-mediated mRNA decay.

Trcek T, Sato H, Singer RH, Maquat LE

Genes & development.. 2013 March 127 (5):541-51. Epub 02/21/2013.

Rules that govern UPF1 binding to mRNA 3' UTRs.

Kurosaki T, Maquat LE

Proceedings of the National Academy of Sciences of the United States of America.. 2013 February 26110 (9):3357-62. Epub 02/12/2013.

Staufen2 functions in Staufen1-mediated mRNA decay by binding to itself and its paralog and promoting UPF1 helicase but not ATPase activity.

Park E, Gleghorn ML, Maquat LE

Proceedings of the National Academy of Sciences of the United States of America.. 2013 January 8110 (2):405-12. Epub 12/20/2012.

Staufen-mediated mRNA decay.

Park E, Maquat LE

Wiley interdisciplinary reviews. RNA.. 2013 4 (4):423-35. Epub 05/16/2013.

Organizing principles of mammalian nonsense-mediated mRNA decay.

Wei-Lin Popp M, Maquat LE

Annual review of genetics. 2013 47 :139-65. Epub 1900 01 01.

Biochemical analysis of long non-coding RNA-containing ribonucleoprotein complexes.

Gong C, Popp MW, Maquat LE

Methods : a companion to Methods in enzymology.. 2012 October 58 (2):88-93. Epub 07/10/2012.

Regulation of cytoplasmic mRNA decay.

Schoenberg DR, Maquat LE

Nature reviews. Genetics.. 2012 March 613 (4):246-59. Epub 03/06/2012.

Nonsense-mediated mRNA decay (NMD) in animal embryogenesis: to die or not to die, that is the question.

Hwang J, Maquat LE

Current opinion in genetics & development.. 2011 August 21 (4):422-30. Epub 1900 01 01.

"Alu"strious long ncRNAs and their role in shortening mRNA half-lives.

Gong C, Maquat LE

Cell cycle.. 2011 June 1510 (12):1882-3. Epub 06/15/2011.

A yeast model of FUS/TLS-dependent cytotoxicity.

Ju S, Tardiff DF, Han H, Divya K, Zhong Q, Maquat LE, Bosco DA, Hayward LJ, Brown RH, Lindquist S, Ringe D, Petsko GA

PLoS biology.. 2011 April 9 (4):e1001052. Epub 04/26/2011.

UPF1 learns to relax and unwind.

Gleghorn ML, Maquat LE

Molecular cell.. 2011 March 1841 (6):621-3. Epub 1900 01 01.

lncRNAs transactivate STAU1-mediated mRNA decay by duplexing with 3' UTRs via Alu elements.

Gong C, Maquat LE

Nature.. 2011 February 10470 (7333):284-8. Epub 1900 01 01.

UPF1 association with the cap-binding protein, CBP80, promotes nonsense-mediated mRNA decay at two distinct steps.

Hwang J, Sato H, Tang Y, Matsuda D, Maquat LE

Molecular cell.. 2010 August 1339 (3):396-409. Epub 08/05/2010.

The pioneer round of translation: features and functions.

Maquat LE, Tarn WY, Isken O

Cell.. 2010 August 6142 (3):368-74. Epub 1900 01 01.

CBP80-promoted mRNP rearrangements during the pioneer round of translation, nonsense-mediated mRNA decay, and thereafter.

Maquat LE, Hwang J, Sato H, Tang Y

Cold Spring Harbor symposia on quantitative biology.. 2010 75 :127-34. Epub 03/29/2011.

Gene expression networks: competing mRNA decay pathways in mammalian cells.

Maquat LE, Gong C

Biochemical Society transactions.. 2009 December 37 (Pt 6):1287-92. Epub 1900 01 01.

Remodeling of the pioneer translation initiation complex involves translation and the karyopherin importin beta.

Sato H, Maquat LE

Genes & development.. 2009 November 123 (21):2537-50. Epub 1900 01 01.

Re-capping the message.

Schoenberg DR, Maquat LE

Trends in biochemical sciences.. 2009 September 34 (9):435-42. Epub 09/02/2009.

SMD and NMD are competitive pathways that contribute to myogenesis: effects on PAX3 and myogenin mRNAs.

Gong C, Kim YK, Woeller CF, Tang Y, Maquat LE

Genes & development.. 2009 January 123 (1):54-66. Epub 12/18/2008.

Telomeric RNAs as a novel player in telomeric integrity.

Isken O, Maquat LE

F1000 biology reports. 2009 1 :90. Epub 11/26/2009.

The multiple lives of NMD factors: balancing roles in gene and genome regulation.

Isken O, Maquat LE

Nature reviews. Genetics.. 2008 September 9 (9):699-712. Epub 1900 01 01.

Regulation of multiple core spliceosomal proteins by alternative splicing-coupled nonsense-mediated mRNA decay.

Saltzman AL, Kim YK, Pan Q, Fagnani MM, Maquat LE, Blencowe BJ

Molecular and cellular biology.. 2008 July 28 (13):4320-30. Epub 04/28/2008.

NMD resulting from encephalomyocarditis virus IRES-directed translation initiation seems to be restricted to CBP80/20-bound mRNA.

Woeller CF, Gaspari M, Isken O, Maquat LE

EMBO reports.. 2008 May 9 (5):446-51. Epub 03/28/2008.

Upf1 phosphorylation triggers translational repression during nonsense-mediated mRNA decay.

Isken O, Kim YK, Hosoda N, Mayeur GL, Hershey JW, Maquat LE

Cell.. 2008 April 18133 (2):314-27. Epub 1900 01 01.

Efficiency of the pioneer round of translation affects the cellular site of nonsense-mediated mRNA decay.

Sato H, Hosoda N, Maquat LE

Molecular cell.. 2008 February 129 (2):255-62. Epub 1900 01 01.

RNA turnover in eukaryotes: nucleases, pathways and analysis of mRNA decay. Preface.

Maquat LE, Kiledjian M

Methods in enzymology.. 2008 448 :xxi-xxii. Epub 1900 01 01.

RNA turnover in prokaryotes, archaea and organelles. Preface.

Maquat LE, Arraiano CM

Methods in enzymology.. 2008 447 :xxiii-xxiv. Epub 1900 01 01.

RNA turnover in eukaryotes: analysis of specialized and quality control RNA decay pathways. Preface.

Maquat LE, Kiledjian M

Methods in enzymology.. 2008 449 :xvii-xviii. Epub 1900 01 01.

Chapter 9. Studying nonsense-mediated mRNA decay in mammalian cells.

Matsuda D, Sato H, Maquat LE

Methods in enzymology.. 2008 449 :177-201. Epub 1900 01 01.

Failsafe nonsense-mediated mRNA decay does not detectably target eIF4E-bound mRNA.

Matsuda D, Hosoda N, Kim YK, Maquat LE

Nature structural & molecular biology.. 2007 October 14 (10):974-9. Epub 09/16/2007.

Quality control of eukaryotic mRNA: safeguarding cells from abnormal mRNA function.

Isken O, Maquat LE

Genes & development.. 2007 August 121 (15):1833-56. Epub 1900 01 01.

Staufen1 regulates diverse classes of mammalian transcripts.

Kim YK, Furic L, Parisien M, Major F, DesGroseillers L, Maquat LE

The EMBO journal.. 2007 June 626 (11):2670-81. Epub 05/17/2007.

Applying nonsense-mediated mRNA decay research to the clinic: progress and challenges.

Kuzmiak HA, Maquat LE

Trends in molecular medicine.. 2006 July 12 (7):306-16. Epub 06/16/2006.

Evidence that poly(A) binding protein C1 binds nuclear pre-mRNA poly(A) tails.

Hosoda N, Lejeune F, Maquat LE

Molecular and cellular biology.. 2006 April 26 (8):3085-97. Epub 1900 01 01.

Quantitative microarray profiling provides evidence against widespread coupling of alternative splicing with nonsense-mediated mRNA decay to control gene expression.

Pan Q, Saltzman AL, Kim YK, Misquitta C, Shai O, Maquat LE, Frey BJ, Blencowe BJ

Genes & development.. 2006 January 1520 (2):153-8. Epub 1900 01 01.

CBP80 promotes interaction of Upf1 with Upf2 during nonsense-mediated mRNA decay in mammalian cells.

Hosoda N, Kim YK, Lejeune F, Maquat LE

Nature structural & molecular biology.. 2005 October 12 (10):893-901. Epub 09/25/2005.

Mechanistic links between nonsense-mediated mRNA decay and pre-mRNA splicing in mammalian cells.

Lejeune F, Maquat LE

Current opinion in cell biology.. 2005 June 17 (3):309-15. Epub 1900 01 01.

Nonsense-mediated mRNA decay in mammals.

Maquat LE

Journal of cell science.. 2005 May 1118 (Pt 9):1773-6. Epub 1900 01 01.

Cap-binding protein 1-mediated and eukaryotic translation initiation factor 4E-mediated pioneer rounds of translation in yeast.

Gao Q, Das B, Sherman F, Maquat LE

Proceedings of the National Academy of Sciences of the United States of America.. 2005 March 22102 (12):4258-63. Epub 03/07/2005.

Mammalian Staufen1 recruits Upf1 to specific mRNA 3'UTRs so as to elicit mRNA decay.

Kim YK, Furic L, Desgroseillers L, Maquat LE

Cell.. 2005 January 28120 (2):195-208. Epub 1900 01 01.

An enemy within: fly reconnaissance deploys an endonuclease to destroy nonsense-containing mRNA.

Valencia-Sánchez MA, Maquat LE

Trends in cell biology.. 2004 November 14 (11):594-7. Epub 1900 01 01.

eIF4G is required for the pioneer round of translation in mammalian cells.

Lejeune F, Ranganathan AC, Maquat LE

Nature structural & molecular biology.. 2004 October 11 (10):992-1000. Epub 09/07/2004.

The mRNA surveillance protein hSMG-1 functions in genotoxic stress response pathways in mammalian cells.

Brumbaugh KM, Otterness DM, Geisen C, Oliveira V, Brognard J, Li X, Lejeune F, Tibbetts RS, Maquat LE, Abraham RT

Molecular cell.. 2004 June 414 (5):585-98. Epub 1900 01 01.

The pioneer translation initiation complex is functionally distinct from but structurally overlaps with the steady-state translation initiation complex.

Chiu SY, Lejeune F, Ranganathan AC, Maquat LE

Genes & development.. 2004 April 118 (7):745-54. Epub 04/01/2004.

Nonsense-mediated mRNA decay: splicing, translation and mRNP dynamics.

Maquat LE

Nature reviews. Molecular cell biology.. 2004 February 5 (2):89-99. Epub 1900 01 01.

Immunopurification and analysis of protein and RNA components of mRNP in mammalian cells.

Lejeune F, Maquat LE

Methods in molecular biology.. 2004 257 :115-24. Epub 1900 01 01.

Nonsense-mediated mRNA decay in mammalian cells involves decapping, deadenylating, and exonucleolytic activities.

Lejeune F, Li X, Maquat LE

Molecular cell.. 2003 September 12 (3):675-87. Epub 1900 01 01.

Post-transcriptional control of gene expression: effectors of mRNA decay.

Arraiano CM, Maquat LE

Molecular microbiology.. 2003 July 49 (1):267-76. Epub 1900 01 01.

Beta -Globin mRNA decay in erythroid cells: UG site-preferred endonucleolytic cleavage that is augmented by a premature termination codon.

Stevens A, Wang Y, Bremer K, Zhang J, Hoepfner R, Antoniou M, Schoenberg DR, Maquat LE

Proceedings of the National Academy of Sciences of the United States of America.. 2002 October 199 (20):12741-6. Epub 09/19/2002.

The exon junction complex is detected on CBP80-bound but not eIF4E-bound mRNA in mammalian cells: dynamics of mRNP remodeling.

Lejeune F, Ishigaki Y, Li X, Maquat LE

The EMBO journal.. 2002 July 121 (13):3536-45. Epub 1900 01 01.

Molecular biology. Skiing toward nonstop mRNA decay.

Maquat LE

Science.. 2002 March 22295 (5563):2221-2. Epub 1900 01 01.

Nonsense-mediated mRNA decay.

Maquat LE

Current biology : CB.. 2002 March 1912 (6):R196-7. Epub 1900 01 01.

RNA-protein interactions: insight into gene function.

Maquat LE

Methods : a companion to Methods in enzymology.. 2002 February 26 (2):93-4. Epub 1900 01 01.

Nonsense-Mediated Decay: Assaying for Effects on Selenoprotein mRNAs

Sun, X.; Maquat, L.E. ;.

In Methods in Enzymology. Protein Sensors of Reactive Oxygen Species: Selenoproteins, Thioredoxin, Thiol Enzymes, and Proteins, eds. H. Sies, L. Packer, Academic Press. 2002; 347(3): 49-57.

Nonsense-mediated decay: assaying for effects on selenoprotein mRNAs.

Sun X, Maquat LE

Methods in enzymology.. 2002 347 :49-57. Epub 1900 01 01.

New ways of initiating translation in eukaryotes.

Schneider R, Agol VI, Andino R, Bayard F, Cavener DR, Chappell SA, Chen JJ, Darlix JL, Dasgupta A, Donzé O, Duncan R, Elroy-Stein O, Farabaugh PJ, Filipowicz W, Gale M, Gehrke L, Goldman E, Groner Y, Harford JB, Hatzglou M, He B, Hellen CU, Hentze MW, Hershey J, Hershey P, Hohn T, Holcik M, Hunter CP, Igarashi K, Jackson R, Jagus R, Jefferson LS, Joshi B, Kaempfer R, Katze M, Kaufman RJ, Kiledjian M, Kimball SR, Kimchi A, Kirkegaard K, Koromilas AE, Krug RM, Kruys V, Lamphear BJ, Lemon S, Lloyd RE, Maquat LE, Martinez-Salas E, Mathews MB, Mauro VP, Miyamoto S, Mohr I, Morris DR, Moss EG, Nakashima N, Palmenberg A, Parkin NT, Pe'ery T, Pelletier J, Peltz S, Pestova TV, Pilipenko EV, Prats AC, Racaniello V, Read GS, Rhoads RE, Richter JD, Rivera-Pomar R, Rouault T, Sachs A, Sarnow P, Scheper GC, Schiff L, Schoenberg DR, Semler BL, Siddiqui A, Skern T, Sonenberg N, Sossin W, Standart N, Tahara SM, Thomas AA, Toulmé JJ, Wilusz J, Wimmer E, Witherell G, Wormington M

Molecular and cellular biology.. 2001 December 21 (23):8238-46. Epub 1900 01 01.

Cloning of a novel phosphatidylinositol kinase-related kinase: characterization of the human SMG-1 RNA surveillance protein.

Denning G, Jamieson L, Maquat LE, Thompson EA, Fields AP

The Journal of biological chemistry.. 2001 June 22276 (25):22709-14. Epub 04/30/2001.

Nonsense-mediated decay of mRNA for the selenoprotein phospholipid hydroperoxide glutathione peroxidase is detectable in cultured cells but masked or inhibited in rat tissues.

Sun X, Li X, Moriarty PM, Henics T, LaDuca JP, Maquat LE

Molecular biology of the cell.. 2001 April 12 (4):1009-17. Epub 1900 01 01.

Quality control of mRNA function.

Maquat LE, Carmichael GG

Cell.. 2001 January 26104 (2):173-6. Epub 1900 01 01.

Identification and characterization of human orthologues to Saccharomyces cerevisiae Upf2 protein and Upf3 protein (Caenorhabditis elegans SMG-4).

Serin G, Gersappe A, Black JD, Aronoff R, Maquat LE

Molecular and cellular biology.. 2001 January 21 (1):209-23. Epub 1900 01 01.

The power of point mutations.

Maquat LE

Nature genetics.. 2001 January 27 (1):5-6. Epub 1900 01 01.

Nonsense-mediated mRNA decay: insights into mechanism from the cellular abundance of human Upf1, Upf2, Upf3, and Upf3X proteins.

Maquat LE, Serin G

Cold Spring Harbor symposia on quantitative biology.. 2001 66 :313-20. Epub 1900 01 01.

The spliceosome deposits multiple proteins 20-24 nucleotides upstream of mRNA exon-exon junctions.

Le Hir H, Izaurralde E, Maquat LE, Moore MJ

The EMBO journal.. 2000 December 1519 (24):6860-9. Epub 1900 01 01.

Nonsense-mediated decay of glutathione peroxidase 1 mRNA in the cytoplasm depends on intron position.

Sun X, Moriarty PM, Maquat LE

The EMBO journal.. 2000 September 119 (17):4734-44. Epub 1900 01 01.

Pre-mRNA splicing alters mRNP composition: evidence for stable association of proteins at exon-exon junctions.

Le Hir H, Moore MJ, Maquat LE

Genes & development.. 2000 May 114 (9):1098-108. Epub 1900 01 01.

Identical germ-line mutations in the triosephosphate isomerase alleles of two brothers are associated with distinct clinical phenotypes.

Valentin C, Cohen-Solal M, Maquat L, Horányi M, Inselt-Kovács M, Hollán S

Comptes rendus de l'Académie des sciences. Série III, Sciences de

At least one intron is required for the nonsense-mediated decay of triosephosphate isomerase mRNA: a possible link between nuclear splicing and cytoplasmic translation.

Zhang J, Sun X, Qian Y, LaDuca JP, Maquat LE

Molecular and cellular biology.. 1998 September 18 (9):5272-83. Epub 1900 01 01.

A mutated human homologue to yeast Upf1 protein has a dominant-negative effect on the decay of nonsense-containing mRNAs in mammalian cells.

Sun X, Perlick HA, Dietz HC, Maquat LE

Proceedings of the National Academy of Sciences of the United States of America.. 1998 August 1895 (17):10009-14. Epub 1900 01 01.

A rule for termination-codon position within intron-containing genes: when nonsense affects RNA abundance.

Nagy E, Maquat LE

Trends in biochemical sciences.. 1998 June 23 (6):198-9. Epub 1900 01 01.

Evidence that translation reinitiation abrogates nonsense-mediated mRNA decay in mammalian cells.

Zhang J, Maquat LE

The EMBO journal.. 1997 February 1716 (4):826-33. Epub 1900 01 01.

Defects in RNA splicing and the consequence of shortened translational reading frames.

Maquat LE

American journal of human genetics.. 1996 August 59 (2):279-86. Epub 1900 01 01.

Lack of an effect of the efficiency of RNA 3'-end formation on the efficiency of removal of either the final or the penultimate intron in intact cells.

Nesic D, Zhang J, Maquat LE

Molecular and cellular biology.. 1995 January 15 (1):488-96. Epub 1900 01 01.

Mammalian nonsense codons can be cis effectors of nuclear mRNA half-life.

Belgrader P, Cheng J, Zhou X, Stephenson LS, Maquat LE

Molecular and cellular biology.. 1994 December 14 (12):8219-28. Epub 1900 01 01.

Introns are cis effectors of the nonsense-codon-mediated reduction in nuclear mRNA abundance.

Cheng J, Belgrader P, Zhou X, Maquat LE

Molecular and cellular biology.. 1994 September 14 (9):6317-25. Epub 1900 01 01.

Upstream introns influence the efficiency of final intron removal and RNA 3'-end formation.

Nesic D, Maquat LE

Genes & development.. 1994 February 18 (3):363-75. Epub 1900 01 01.

Sequences within the last intron function in RNA 3'-end formation in cultured cells.

Nesic D, Cheng J, Maquat LE

Molecular and cellular biology.. 1993 June 13 (6):3359-69. Epub 1900 01 01.

Human triosephosphate isomerase deficiency resulting from mutation of Phe-240.

Chang ML, Artymiuk PJ, Wu X, Hollán S, Lammi A, Maquat LE

American journal of human genetics.. 1993 June 52 (6):1260-9. Epub 1900 01 01.

Nonsense codons can reduce the abundance of nuclear mRNA without affecting the abundance of pre-mRNA or the half-life of cytoplasmic mRNA.

Cheng J, Maquat LE

Molecular and cellular biology.. 1993 March 13 (3):1892-902. Epub 1900 01 01.

Evidence to implicate translation by ribosomes in the mechanism by which nonsense codons reduce the nuclear level of human triosephosphate isomerase mRNA.

Belgrader P, Cheng J, Maquat LE

Proceedings of the National Academy of Sciences of the United States of America.. 1993 January 1590 (2):482-6. Epub 1900 01 01.

Nonsense codons in human beta-globin mRNA result in the production of mRNA degradation products.

Lim SK, Sigmund CD, Gross KW, Maquat LE

Molecular and cellular biology.. 1992 March 12 (3):1149-61. Epub 1900 01 01.

Nuclear mRNA export.

Maquat LE

Current opinion in cell biology.. 1991 December 3 (6):1004-12. Epub 1900 01 01.

Modulation of human triosephosphate isomerase gene transcription by serum.

Boyer TG, Maquat LE

The Journal of biological chemistry.. 1991 July 15266 (20):13350-4. Epub 1900 01 01.

Minimal sequence and factor requirements for the initiation of transcription from an atypical, TATATAA box-containing housekeeping promoter.

Boyer TG, Maquat LE

The Journal of biological chemistry.. 1990 November 25265 (33):20524-32. Epub 1900 01 01.

Translation to near the distal end of the penultimate exon is required for normal levels of spliced triosephosphate isomerase mRNA.

Cheng J, Fogel-Petrovic M, Maquat LE

Molecular and cellular biology.. 1990 October 10 (10):5215-25. Epub 1900 01 01.

Nucleotide sequence of murine triosephosphate isomerase cDNA.

Cheng J, Mielnicki LM, Pruitt SC, Maquat LE

Nucleic acids research.. 1990 July 2518 (14):4261. Epub 1900 01 01.

Novel metabolism of several beta zero-thalassemic beta-globin mRNAs in the erythroid tissues of transgenic mice.

Lim S, Mullins JJ, Chen CM, Gross KW, Maquat LE

The EMBO journal.. 1989 September 8 (9):2613-9. Epub 1900 01 01.

Transcriptional regulatory sequences of the housekeeping gene for human triosephosphate isomerase.

Boyer TG, Krug JR, Maquat LE

The Journal of biological chemistry.. 1989 March 25264 (9):5177-87. Epub 1900 01 01.

Premature translation termination mediates triosephosphate isomerase mRNA degradation.

Daar IO, Maquat LE

Molecular and cellular biology.. 1988 February 8 (2):802-13. Epub 1900 01 01.

Human triose-phosphate isomerase deficiency: a single amino acid substitution results in a thermolabile enzyme.

Daar IO, Artymiuk PJ, Phillips DC, Maquat LE

Proceedings of the National Academy of Sciences of the United States of America.. 1986 October 83 (20):7903-7. Epub 1900 01 01.

Localization of DNA sequences involved in dexamethasone-dependent expression of the rat alpha 1-acid glycoprotein gene.

Baumann H, Maquat LE

Molecular and cellular biology.. 1986 July 6 (7):2551-61. Epub 1900 01 01.

Characterization of the functional gene and several processed pseudogenes in the human triosephosphate isomerase gene family.

Brown JR, Daar IO, Krug JR, Maquat LE

Molecular and cellular biology.. 1985 July 5 (7):1694-706. Epub 1900 01 01.

A beta zero-thalassemic beta-globin RNA that is labile in bone marrow cells is relatively stable in HeLa cells.

Maquat LE, Kinniburgh AJ

Nucleic acids research.. 1985 April 2513 (8):2855-67. Epub 1900 01 01.

Human triosephosphate isomerase cDNA and protein structure. Studies of triosephosphate isomerase deficiency in man.

Maquat LE, Chilcote R, Ryan PM

The Journal of biological chemistry.. 1985 March 25260 (6):3748-53. Epub 1900 01 01.

mRNA-deficient beta o-thalassemia results from a single nucleotide deletion.

Kinniburgh AJ, Maquat LE, Schedl T, Rachmilewitz E, Ross J

Nucleic acids research.. 1982 September 2510 (18):5421-7. Epub 1900 01 01.

Unstable beta-globin mRNA in mRNA-deficient beta o thalassemia.

Maquat LE, Kinniburgh AJ, Rachmilewitz EA, Ross J

Cell.. 1981 December 27 (3 Pt 2):543-53. Epub 1900 01 01.

Processing of human beta-globin mRNA precursor to mRNA is defective in three patients with beta+-thalassemia.

Maquat LE, Kinniburgh AJ, Beach LR, Honig GR, Lazerson J, Ershler WB, Ross J

Proceedings of the National Academy of Sciences of the United States of America.. 1980 July 77 (7):4287-91. Epub 1900 01 01.

lac Promoter mutation Pr115 generates a new transcription initiation point.

Maquat LE, Reznikoff WS

Journal of molecular biology.. 1980 May 25139 (3):551-6. Epub 1900 01 01.

lac Promoter mutations located downstream from the transcription start site.

Maquat LE, Thornton K, Reznikoff WS

Journal of molecular biology.. 1980 May 25139 (3):537-49. Epub 1900 01 01.

Influence of A-T content on the fractionation of DNA restriction fragments by RPC-5 column chromatography.

Patient RK, Hardies SC, Larson JE, Inman RB, Maquat LE, Wells RD

The Journal of biological chemistry.. 1979 June 25254 (12):5548-54. Epub 1900 01 01.

In vitro analysis of the Escherichia coli RNA polymerase interaction with wild-type and mutant lactose promoters.

Maquat LE, Reznikoff WS

Journal of molecular biology.. 1978 November 15125 (4):467-90. Epub 1900 01 01.