Vamsi K Mootha

Vamsi K Mootha

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Leigh Syndrome Mouse Model Can Be Rescued by Interventions that Normalize Brain Hyperoxia, but Not HIF Activation.

Cell Metab 2019 Oct 8;30(4):824-832.e3. Epub 2019 Aug 8.

Department of Molecular Biology and Howard Hughes Medical Institute, Massachusetts General Hospital, Boston, MA, USA; Department of Systems Biology, Harvard Medical School, Boston, MA, USA; Broad Institute of Harvard and MIT, Cambridge, MA, USA. Electronic address:

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http://dx.doi.org/10.1016/j.cmet.2019.07.006DOI Listing
October 2019

Mitochondrial Reprogramming Underlies Resistance to BCL-2 Inhibition in Lymphoid Malignancies.

Cancer Cell 2019 Oct 19;36(4):369-384.e13. Epub 2019 Sep 19.

Department of Medical Oncology, Dana-Farber Cancer Institute, 450 Brookline Avenue, Dana Building, Room DA-520, Boston MA 02215-02115, USA; Broad Institute of MIT and Harvard, Cambridge, MA 02142, USA; Harvard Medical School, Boston, MA 02215, USA; Department of Medicine, Brigham and Women's Hospital, Boston, MA 02215, USA. Electronic address:

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http://dx.doi.org/10.1016/j.ccell.2019.08.005DOI Listing
October 2019

Epstein-Barr-Virus-Induced One-Carbon Metabolism Drives B Cell Transformation.

Cell Metab 2019 Sep 27;30(3):539-555.e11. Epub 2019 Jun 27.

Graduate Program in Virology, Division of Medical Sciences, Harvard Medical School, 77 Avenue Louis Pasteur, Boston, MA 02115, USA; Division of Infectious Diseases, Department of Medicine, Brigham and Women's Hospital, 181 Longwood Avenue, Boston, MA 02115, USA; Broad Institute of Harvard and MIT, Cambridge, MA 02142, USA; Department of Microbiology, Harvard Medical School, Boston, MA 02115, USA. Electronic address:

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http://dx.doi.org/10.1016/j.cmet.2019.06.003DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6720460PMC
September 2019

Hypoxia Rescues Frataxin Loss by Restoring Iron Sulfur Cluster Biogenesis.

Cell 2019 May 25;177(6):1507-1521.e16. Epub 2019 Apr 25.

Broad Institute, Cambridge, MA 02142, USA; Howard Hughes Medical Institute, Howard Hughes Medical Institute, Massachusetts General Hospital, Boston, MA 02114, USA; Department of Molecular Biology, Massachusetts General Hospital, Boston, MA 02114, USA; Harvard Medical School, Boston, MA 02115, USA. Electronic address:

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http://dx.doi.org/10.1016/j.cell.2019.03.045DOI Listing
May 2019

Exploring the In Vivo Role of the Mitochondrial Calcium Uniporter in Brown Fat Bioenergetics.

Cell Rep 2019 Apr;27(5):1364-1375.e5

Howard Hughes Medical Institute and Department of Molecular Biology, Massachusetts General Hospital, Boston, MA 02114, USA; Department of Systems Biology, Harvard Medical School, Boston, MA 02115, USA; Broad Institute, Cambridge, MA 02141, USA. Electronic address:

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http://dx.doi.org/10.1016/j.celrep.2019.04.013DOI Listing
April 2019

Impaired hypoxic pulmonary vasoconstriction in a mouse model of Leigh syndrome.

Am J Physiol Lung Cell Mol Physiol 2019 Feb 6;316(2):L391-L399. Epub 2018 Dec 6.

Anesthesia Center for Critical Care Research of the Department of Anesthesia, Critical Care, and Pain Medicine, Harvard Medical School and Massachusetts General Hospital, Boston, Massachusetts.

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https://www.physiology.org/doi/10.1152/ajplung.00419.2018
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http://dx.doi.org/10.1152/ajplung.00419.2018DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6397345PMC
February 2019

Crystal structure of MICU2 and comparison with MICU1 reveal insights into the uniporter gating mechanism.

Proc Natl Acad Sci U S A 2019 02 12;116(9):3546-3555. Epub 2019 Feb 12.

Howard Hughes Medical Institute, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114;

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http://dx.doi.org/10.1073/pnas.1817759116DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6397551PMC
February 2019

Evolutionary mitochondrial biology in titisee.

IUBMB Life 2018 12 24;70(12):1184-1187. Epub 2018 Oct 24.

Howard Hughes Medical Institute and Department of Molecular Biology, Massachsuetts General Hospital, Boston, MA, USA.

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http://doi.wiley.com/10.1002/iub.1958
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http://dx.doi.org/10.1002/iub.1958DOI Listing
December 2018

Author Correction: Cryo-EM structure of a fungal mitochondrial calcium uniporter.

Nature 2018 10;562(7728):E25

Howard Hughes Medical Institute and Department of Physiology, University of Texas Southwestern Medical Center, Dallas, TX, USA.

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http://dx.doi.org/10.1038/s41586-018-0427-1DOI Listing
October 2018

Oxygen in mitochondrial disease: can there be too much of a good thing?

J Inherit Metab Dis 2018 09 8;41(5):761-763. Epub 2018 Jun 8.

MRC Mitochondrial Biology Unit & Department of Clinical Neurosciences, University of Cambridge, Cambridge Biomedical Campus, Cambridge, CB2 0QQ, UK.

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http://dx.doi.org/10.1007/s10545-018-0210-3DOI Listing
September 2018

MICU1 imparts the mitochondrial uniporter with the ability to discriminate between Ca and Mn.

Proc Natl Acad Sci U S A 2018 08 6;115(34):E7960-E7969. Epub 2018 Aug 6.

Howard Hughes Medical Institute, Massachusetts General Hospital, Boston, MA 02114;

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http://dx.doi.org/10.1073/pnas.1807811115DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6112746PMC
August 2018

Widespread Chromosomal Losses and Mitochondrial DNA Alterations as Genetic Drivers in Hürthle Cell Carcinoma.

Cancer Cell 2018 08;34(2):242-255.e5

Department of Medicine, Massachusetts General Hospital, Boston, MA 02114, USA; Thyroid Unit, Massachusetts General Hospital, Boston, MA 02114, USA; Department of Internal Medicine, Division of Endocrinology, University of Texas Southwestern Medical Center, Dallas, TX 75390, USA; Department of Biochemistry, University of Texas Southwestern Medical Center, Dallas, TX 75390, USA; Harold C. Simmons Comprehensive Cancer Center, University of Texas Southwestern Medical Center, Dallas, TX 75390, USA. Electronic address:

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http://dx.doi.org/10.1016/j.ccell.2018.06.013DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6121811PMC
August 2018

Early loss of mitochondrial complex I and rewiring of glutathione metabolism in renal oncocytoma.

Proc Natl Acad Sci U S A 2018 07 18;115(27):E6283-E6290. Epub 2018 Jun 18.

Howard Hughes Medical Institute and Department of Molecular Biology, Massachusetts General Hospital, Boston, MA 02114;

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http://dx.doi.org/10.1073/pnas.1711888115DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6142220PMC
July 2018

Cryo-EM structure of a fungal mitochondrial calcium uniporter.

Nature 2018 07 11;559(7715):570-574. Epub 2018 Jul 11.

Howard Hughes Medical Institute and Department of Physiology, University of Texas Southwestern Medical Center, Dallas, TX, USA.

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http://dx.doi.org/10.1038/s41586-018-0333-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6063787PMC
July 2018

Spatiotemporal compartmentalization of hepatic NADH and NADPH metabolism.

J Biol Chem 2018 05 7;293(20):7508-7516. Epub 2018 Mar 7.

Howard Hughes Medical Institute, and Department of Molecular Biology, Massachusetts General Hospital, Boston, Massachusetts 02114 and

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http://dx.doi.org/10.1074/jbc.TM117.000258DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5961030PMC
May 2018

Antibodies to biotin enable large-scale detection of biotinylation sites on proteins.

Nat Methods 2017 Dec 16;14(12):1167-1170. Epub 2017 Oct 16.

Proteomics, Broad Institute of MIT and Harvard, Cambridge, Massachusetts, USA.

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http://dx.doi.org/10.1038/nmeth.4465DOI Listing
December 2017

The Human Knockout Gene CLYBL Connects Itaconate to Vitamin B.

Cell 2017 Nov 19;171(4):771-782.e11. Epub 2017 Oct 19.

Howard Hughes Medical Institute and Department of Molecular Biology, Massachusetts General Hospital, Boston, MA 02114, USA; Department of Systems Biology, Harvard Medical School, Boston, MA 02115, USA; Broad Institute, Cambridge, MA 02141, USA. Electronic address:

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http://dx.doi.org/10.1016/j.cell.2017.09.051DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5827971PMC
November 2017

A genetically encoded tool for manipulation of NADP/NADPH in living cells.

Nat Chem Biol 2017 Oct 7;13(10):1088-1095. Epub 2017 Aug 7.

Howard Hughes Medical Institute and Department of Molecular Biology, Massachusetts General Hospital, Boston, Massachusetts, USA.

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http://www.nature.com/doifinder/10.1038/nchembio.2454
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http://dx.doi.org/10.1038/nchembio.2454DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5605434PMC
October 2017

High-affinity cooperative Ca binding by MICU1-MICU2 serves as an on-off switch for the uniporter.

EMBO Rep 2017 08 14;18(8):1397-1411. Epub 2017 Jun 14.

Howard Hughes Medical Institute and Department of Molecular Biology, Massachusetts General Hospital, Boston, MA, USA

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http://dx.doi.org/10.15252/embr.201643748DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5538426PMC
August 2017

Metabolic profiles of exercise in patients with McArdle disease or mitochondrial myopathy.

Proc Natl Acad Sci U S A 2017 08 17;114(31):8402-8407. Epub 2017 Jul 17.

Howard Hughes Medical Institute, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114;

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http://dx.doi.org/10.1073/pnas.1703338114DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5547614PMC
August 2017

Natural Product Screening Reveals Naphthoquinone Complex I Bypass Factors.

PLoS One 2016 13;11(9):e0162686. Epub 2016 Sep 13.

Howard Hughes Medical Institute and Department of Molecular Biology, Massachusetts General Hospital, Boston, MA, United States of America.

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0162686PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5021346PMC
July 2017

CLIC, a tool for expanding biological pathways based on co-expression across thousands of datasets.

PLoS Comput Biol 2017 Jul 18;13(7):e1005653. Epub 2017 Jul 18.

Howard Hughes Medical Institute and Department of Molecular Biology and the Center for Genomic Medicine, Massachusetts General Hospital, Boston, MA, United States of America and Department of Systems Biology, Harvard Medical School, Boston, MA United States of America.

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http://dx.doi.org/10.1371/journal.pcbi.1005653DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5546725PMC
July 2017

Hypoxia treatment reverses neurodegenerative disease in a mouse model of Leigh syndrome.

Proc Natl Acad Sci U S A 2017 05 8;114(21):E4241-E4250. Epub 2017 May 8.

Department of Anesthesia, Critical Care, and Pain Medicine, Massachusetts General Hospital, Boston, MA 02114;

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http://dx.doi.org/10.1073/pnas.1621511114DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5448167PMC
May 2017

Comparative Analysis of Mitochondrial N-Termini from Mouse, Human, and Yeast.

Mol Cell Proteomics 2017 04 25;16(4):512-523. Epub 2017 Jan 25.

From the ‡Howard Hughes Medical Institute, Department of Molecular Biology, Massachusetts General Hospital, Boston, Massachusetts 02114.

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http://dx.doi.org/10.1074/mcp.M116.063818DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5383775PMC
April 2017

A Genome-wide CRISPR Death Screen Identifies Genes Essential for Oxidative Phosphorylation.

Cell Metab 2016 12 22;24(6):875-885. Epub 2016 Sep 22.

Department of Molecular Biology and Howard Hughes Medical Institute, Massachusetts General Hospital, Boston, MA 02114, USA; Department of Systems Biology, Harvard Medical School, Boston, MA 02115, USA; Broad Institute of MIT and Harvard, Cambridge, MA 02142, USA. Electronic address:

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http://www.cell.com/cell-metabolism/pdf/S1550-4131(16)30433-
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http://www.cell.com/cms/attachment/2064501967/2065983246/mmc
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http://dx.doi.org/10.1016/j.cmet.2016.08.017DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5474757PMC
December 2016

Natural underlying mtDNA heteroplasmy as a potential source of intra-person hiPSC variability.

EMBO J 2016 09 19;35(18):1979-90. Epub 2016 Jul 19.

Departments of Cardiovascular Diseases, Molecular Pharmacology and Experimental Therapeutics, Division of General Internal Medicine, Division of Pediatric Cardiology, and Transplant Center, Mayo Clinic Center for Regenerative Medicine, Rochester, MN, USA

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http://dx.doi.org/10.15252/embj.201694892DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5282833PMC
September 2016

Architecture of the mitochondrial calcium uniporter.

Nature 2016 05 2;533(7602):269-73. Epub 2016 May 2.

Department of Biological Chemistry and Molecular Pharmacology, Harvard Medical School, Boston, Massachusetts 02115, USA.

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http://dx.doi.org/10.1038/nature17656DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4874835PMC
May 2016

Hypoxia as a therapy for mitochondrial disease.

Science 2016 Apr 25;352(6281):54-61. Epub 2016 Feb 25.

Department of Molecular Biology and Howard Hughes Medical Institute, Massachusetts General Hospital, Boston, MA, USA. Department of Systems Biology, Harvard Medical School, Boston, MA, USA. Broad Institute of Harvard and MIT, Cambridge, MA, USA.

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http://dx.doi.org/10.1126/science.aad9642DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4860742PMC
April 2016

Homozygous deletion in MICU1 presenting with fatigue and lethargy in childhood.

Neurol Genet 2016 Apr 3;2(2):e59. Epub 2016 Mar 3.

Wellcome Trust Centre for Mitochondrial Research (D.L.-S., H.G., J.D., A.P., R.W.T., P.Y.-W.-M., R.H., P.F.C.), Institute of Genetic Medicine (D.L.-S., H.G., J.D., A.P., P.Y.-W.-M., R.H.), and Institute of Neuroscience (R.W.T.), Newcastle University, Newcastle upon Tyne, United Kingdom; Howard Hughes Medical Institute (K.J.K., D.T., V.K.M.), Department of Molecular Biology, Massachusetts General Hospital, Boston, MA; Department of Paediatric Neurology (A.-M.C., K.P.), The General Infirmary, Leeds, United Kingdom; Department of Child Neurology (V.R.), The Royal Victoria Infirmary, Newcastle upon Tyne Hospitals NHS Trust, Newcastle upon Tyne, United Kingdom; Department of Systems Biology (V.K.M.), Harvard Medical School, Boston, MA; Broad Institute (V.K.M.), Cambridge, MA; Department of Clinical Neurosciences (P.F.C.), University of Cambridge; and MRC Mitochondrial Biology Unit (P.F.C.), Cambridge Biomedical Campus, United Kingdom.

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http://dx.doi.org/10.1212/NXG.0000000000000059DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4830195PMC
April 2016

Complementation of mitochondrial electron transport chain by manipulation of the NAD+/NADH ratio.

Science 2016 Apr 7;352(6282):231-5. Epub 2016 Apr 7.

Howard Hughes Medical Institute and Department of Molecular Biology, Massachusetts General Hospital, Boston, MA, USA. Department of Systems Biology, Harvard Medical School, Boston, MA, USA. Broad Institute, Cambridge, MA, USA.

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http://dx.doi.org/10.1126/science.aad4017DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4850741PMC
April 2016

MitoCarta2.0: an updated inventory of mammalian mitochondrial proteins.

Nucleic Acids Res 2016 Jan 7;44(D1):D1251-7. Epub 2015 Oct 7.

Howard Hughes Medical Institute and Department of Molecular Biology, Massachusetts General Hospital, Boston, MA 02114, USA Broad Institute, Cambridge, MA 02141, USA Department of Systems Biology, Harvard Medical School, Boston, MA 02115, USA

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http://dx.doi.org/10.1093/nar/gkv1003DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4702768PMC
January 2016

Metabolic enzyme expression highlights a key role for MTHFD2 and the mitochondrial folate pathway in cancer.

Nat Commun 2014 ;5:3128

1] Broad Institute, Cambridge, Massachusetts 02142, USA [2] Department of Systems Biology, Harvard Medical School, Boston, Massachusetts 02115, USA [3] Department of Molecular Biology and the Center for Human Genetic Research, Massachusetts General Hospital, Boston, Massachusetts 02114, USA.

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http://dx.doi.org/10.1038/ncomms4128DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4106362PMC
October 2015

The molecular era of the mitochondrial calcium uniporter.

Nat Rev Mol Cell Biol 2015 Sep 19;16(9):545-53. Epub 2015 Aug 19.

Howard Hughes Medical Institute and Department of Molecular Biology, Massachusetts General Hospital, 185 Cambridge Street 6th Floor, Boston, Massachusetts 02114, USA.

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http://dx.doi.org/10.1038/nrm4039DOI Listing
September 2015

Meclizine Preconditioning Protects the Kidney Against Ischemia-Reperfusion Injury.

EBioMedicine 2015 Sep 29;2(9):1090-101. Epub 2015 Jul 29.

Renal Division, Department of Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA 02115, USA ; Harvard Stem Cell Institute, Cambridge, MA 02138, USA.

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http://dx.doi.org/10.1016/j.ebiom.2015.07.035DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4588407PMC
September 2015

IGF2BP2/IMP2-Deficient mice resist obesity through enhanced translation of Ucp1 mRNA and Other mRNAs encoding mitochondrial proteins.

Cell Metab 2015 Apr;21(4):609-21

Department of Molecular Biology, Massachusetts General Hospital, Boston, MA 02114, USA; Diabetes Unit, Medical Services, Massachusetts General Hospital, Boston, MA 02114, USA; Department of Medicine, Harvard Medical School, Boston, MA 02115, USA. Electronic address:

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http://www.cell.com/cms/attachment/2035263924/2050689197/mmc
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http://linkinghub.elsevier.com/retrieve/pii/S155041311500109
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http://dx.doi.org/10.1016/j.cmet.2015.03.006DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4663978PMC
April 2015

Mutation in the novel nuclear-encoded mitochondrial protein CHCHD10 in a family with autosomal dominant mitochondrial myopathy.

Neurogenetics 2015 Jan 6;16(1):1-9. Epub 2014 Sep 6.

Division of Neuromuscular Medicine, The Ken and Ruth Davee Department of Neurology and Clinical Neurosciences, Feinberg School of Medicine, Northwestern University, Chicago, IL, 60611, USA,

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http://link.springer.com/10.1007/s10048-014-0421-1
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http://dx.doi.org/10.1007/s10048-014-0421-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4796476PMC
January 2015

Directed evolution of APEX2 for electron microscopy and proximity labeling.

Nat Methods 2015 Jan 24;12(1):51-4. Epub 2014 Nov 24.

Department of Chemistry, Massachusetts Institute of Technology, Cambridge, Massachusetts, USA.

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http://dx.doi.org/10.1038/nmeth.3179DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4296904PMC
January 2015

Effects of sodium benzoate, a widely used food preservative, on glucose homeostasis and metabolic profiles in humans.

Mol Genet Metab 2015 Jan 15;114(1):73-9. Epub 2014 Nov 15.

Massachusetts General Hospital, Boston MA 02114, USA; Broad Institute of Harvard and MIT, Cambridge, MA 02142, USA. Electronic address:

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http://dx.doi.org/10.1016/j.ymgme.2014.11.010DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4289147PMC
January 2015

Biochemical characterization of pathogenic mutations in human mitochondrial methionyl-tRNA formyltransferase.

J Biol Chem 2014 Nov 6;289(47):32729-41. Epub 2014 Oct 6.

From the Department of Biology, Massachusetts Institute of Technology, Cambridge, Massachusetts 02139,

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http://dx.doi.org/10.1074/jbc.M114.610626DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4239624PMC
November 2014

The uniporter: from newly identified parts to function.

Biochem Biophys Res Commun 2014 Jul 9;449(4):370-2. Epub 2014 May 9.

Howard Hughes Medical Institute, Department of Molecular Biology, Massachusetts General Hospital, Boston, MA 02114, USA; Department of Systems Biology, Harvard Medical School, Boston, MA 02115, USA; Broad Institute, Cambridge, MA 02142, USA. Electronic address:

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http://dx.doi.org/10.1016/j.bbrc.2014.04.143DOI Listing
July 2014

Expansion of biological pathways based on evolutionary inference.

Cell 2014 Jul;158(1):213-25

Howard Hughes Medical Institute and Department of Molecular Biology, Massachusetts General Hospital, Boston, MA 02114, USA; Broad Institute, Cambridge, MA 02141, USA; Department of Systems Biology, Harvard Medical School, Boston, MA 02115, USA. Electronic address:

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http://dx.doi.org/10.1016/j.cell.2014.05.034DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4171950PMC
July 2014

Proteomic mapping of the human mitochondrial intermembrane space in live cells via ratiometric APEX tagging.

Mol Cell 2014 Jul 4;55(2):332-41. Epub 2014 Jul 4.

Department of Chemistry, Massachusetts Institute of Technology, Cambridge, MA 02139, USA; Broad Institute of MIT and Harvard, Cambridge, MA 02142, USA. Electronic address:

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http://dx.doi.org/10.1016/j.molcel.2014.06.003DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4743503PMC
July 2014

Reconstitution of the mitochondrial calcium uniporter in yeast.

Proc Natl Acad Sci U S A 2014 Jun 2;111(24):8985-90. Epub 2014 Jun 2.

Howard Hughes Medical Institute andDepartment of Molecular Biology, Massachusetts General Hospital, Boston, MA 02114;Department of Systems Biology, Harvard Medical School, Boston, MA 02446;Center for Human Genetic Research, Massachusetts General Hospital, Boston, MA 02114; andBroad Institute, Cambridge, MA 02142

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http://dx.doi.org/10.1073/pnas.1400514111DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4066498PMC
June 2014

CLYBL is a polymorphic human enzyme with malate synthase and β-methylmalate synthase activity.

Hum Mol Genet 2014 May 11;23(9):2313-23. Epub 2013 Dec 11.

Department of Molecular Biology, Howard Hughes Medical Institute, Massachusetts General Hospital, Boston, MA 02114, USA.

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http://dx.doi.org/10.1093/hmg/ddt624DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3976331PMC
May 2014

Functional genomic analysis of human mitochondrial RNA processing.

Cell Rep 2014 May 18;7(3):918-31. Epub 2014 Apr 18.

Howard Hughes Medical Institute, Department of Molecular Biology, and Center for Human Genetic Research, Massachusetts General Hospital, Boston, MA 02114, USA; Department of Systems Biology, Harvard Medical School, Boston, MA 02115, USA; Broad Institute, Cambridge, MA 02141, USA. Electronic address:

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http://dx.doi.org/10.1016/j.celrep.2014.03.035DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4289146PMC
May 2014

A systematic survey of lipids across mouse tissues.

Am J Physiol Endocrinol Metab 2014 Apr 11;306(8):E854-68. Epub 2014 Feb 11.

Broad Institute, Cambridge, Massachusetts;

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http://dx.doi.org/10.1152/ajpendo.00371.2013DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3989739PMC
April 2014

MICU1 and MICU2 play nonredundant roles in the regulation of the mitochondrial calcium uniporter.

EMBO Rep 2014 Mar 6;15(3):299-307. Epub 2014 Feb 6.

Department of Molecular Biology, Howard Hughes Medical Institute Massachusetts General Hospital, Boston, MA, USA.

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http://dx.doi.org/10.1002/embr.201337946DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3989696PMC
March 2014

Next generation sequencing with copy number variant detection expands the phenotypic spectrum of HSD17B4-deficiency.

BMC Med Genet 2014 Mar 6;15:30. Epub 2014 Mar 6.

Ataxia Unit, Cognitive Behavioral Neurology Unit, Laboratory for Neuroanatomy and Cerebellar Neurobiology, Department of Neurology, Massachusetts General Hospital & Harvard Medical School, Boston, MA 02114 USA,

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http://dx.doi.org/10.1186/1471-2350-15-30DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4015298PMC
March 2014

Meclizine inhibits mitochondrial respiration through direct targeting of cytosolic phosphoethanolamine metabolism.

J Biol Chem 2013 Dec 19;288(49):35387-95. Epub 2013 Oct 19.

From the Departments of Molecular Biology and Medicine, Center for Human Genetic Research, Massachusetts General Hospital, Boston, Massachusetts 02114.

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http://dx.doi.org/10.1074/jbc.M113.489237DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3853286PMC
December 2013

EMRE is an essential component of the mitochondrial calcium uniporter complex.

Science 2013 Dec 14;342(6164):1379-82. Epub 2013 Nov 14.

Department of Molecular Biology, Massachusetts General Hospital, Department of Systems Biology, Harvard Medical School, Boston, MA, USA.

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http://dx.doi.org/10.1126/science.1242993DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4091629PMC
December 2013

Medicine. A common pathway for a rare disease?

Science 2013 Dec;342(6165):1453-4

Howard Hughes Medical Institute, Departments of Molecular Biology and Medicine, Massachusetts General Hospital, Boston, MA 02114, USA.

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http://dx.doi.org/10.1126/science.1248449DOI Listing
December 2013

Mitochondrial encephalomyopathy due to a novel mutation in ACAD9.

JAMA Neurol 2013 Sep;70(9):1177-9

Department of Neurology, Columbia University Medical Center, College of Physicians and Surgeons, New York, New York2Human Genetics, Joint PhD Program, Universities of Turin and Bologna, Italy.

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http://dx.doi.org/10.1001/jamaneurol.2013.3197DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3891824PMC
September 2013

Distilling pathophysiology from complex disease genetics.

Cell 2013 Sep;155(1):21-6

Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA. Electronic address:

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http://dx.doi.org/10.1016/j.cell.2013.09.001DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4244836PMC
September 2013

New treatments for mitochondrial disease-no time to drop our standards.

Nat Rev Neurol 2013 Aug 2;9(8):474-81. Epub 2013 Jul 2.

Wellcome Trust Centre for Mitochondrial Research, Institute of Genetic Medicine, Ageing and Health, Newcastle University, Newcastle upon Tyne NE1 3BZ, UK.

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http://dx.doi.org/10.1038/nrneurol.2013.129DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4967498PMC
August 2013

MCU encodes the pore conducting mitochondrial calcium currents.

Elife 2013 Jun 4;2:e00704. Epub 2013 Jun 4.

Cardiovascular Research Center , Massachusetts General Hospital , Boston , United States ; Department of Cardiology , Howard Hughes Medical Institute, Boston Children's Hospital , Boston , United States.

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http://dx.doi.org/10.7554/eLife.00704DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3673318PMC
June 2013

Proteomic mapping of mitochondria in living cells via spatially restricted enzymatic tagging.

Science 2013 Mar 31;339(6125):1328-1331. Epub 2013 Jan 31.

Department of Chemistry, Massachusetts Institute of Technology, Cambridge, MA 02139, USA.

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http://dx.doi.org/10.1126/science.1230593DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3916822PMC
March 2013

Complementary RNA and protein profiling identifies iron as a key regulator of mitochondrial biogenesis.

Cell Rep 2013 Jan 10;3(1):237-45. Epub 2013 Jan 10.

Department of Biochemistry, University of Wisconsin-Madison, Madison, WI 53706, USA.

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http://dx.doi.org/10.1016/j.celrep.2012.11.029DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3812070PMC
January 2013

Engineered ascorbate peroxidase as a genetically encoded reporter for electron microscopy.

Nat Biotechnol 2012 Nov 21;30(11):1143-8. Epub 2012 Oct 21.

Department of Chemistry, Massachusetts Institute of Technology, Cambridge, Massachusetts, USA.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3699407PMC
http://dx.doi.org/10.1038/nbt.2375DOI Listing
November 2012

Mitochondrial disorders as windows into an ancient organelle.

Nature 2012 Nov;491(7424):374-83

Department of Molecular Biology, Center for Human Genetic Research, Massachusetts General Hospital, Boston, Massachusetts 02114, USA.

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http://dx.doi.org/10.1038/nature11707DOI Listing
November 2012

Evolutionary diversity of the mitochondrial calcium uniporter.

Science 2012 May;336(6083):886

Department of Molecular Biology, Massachusetts General Hospital, Boston, MA 02114, USA.

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http://dx.doi.org/10.1126/science.1214977DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3518847PMC
May 2012

Meclizine is neuroprotective in models of Huntington's disease.

Hum Mol Genet 2011 Jan 25;20(2):294-300. Epub 2010 Oct 25.

Center for Human Genetic Research, Massachusetts General Hospital, Boston, MA 02114, USA.

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http://dx.doi.org/10.1093/hmg/ddq464DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3005902PMC
January 2011

Neuraminidase inhibitors for influenza in healthy adults: what we don't know.

Natl Med J India 2010 Jan-Feb;23(1):29-31

Department of Pulmonary Medicine, Posgraduate Institute of Medical Education and Research, Chandigarh.

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October 2010

The mitochondrial proteome and human disease.

Annu Rev Genomics Hum Genet 2010 ;11:25-44

Broad Institute of MIT and Harvard, Cambridge, Massachusetts 02142, USA.

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http://dx.doi.org/10.1146/annurev-genom-082509-141720DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4397899PMC
September 2010

MICU1 encodes a mitochondrial EF hand protein required for Ca(2+) uptake.

Nature 2010 Sep 8;467(7313):291-6. Epub 2010 Aug 8.

Center for Human Genetic Research, Massachusetts General Hospital, Boston, Massachusetts 02114, USA.

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http://dx.doi.org/10.1038/nature09358DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2977980PMC
September 2010

Common inherited variation in mitochondrial genes is not enriched for associations with type 2 diabetes or related glycemic traits.

PLoS Genet 2010 Aug 12;6(8). Epub 2010 Aug 12.

Broad Institute of Harvard and Massachusetts Institute of Technology, Cambridge, Massachusetts, United States of America.

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http://dx.doi.org/10.1371/journal.pgen.1001058DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2920848PMC
August 2010

Mitochondrial and nuclear genomic responses to loss of LRPPRC expression.

J Biol Chem 2010 Apr 10;285(18):13742-7. Epub 2010 Mar 10.

Center for Human Genetic Research, Massachusetts General Hospital, Boston, Massachusetts 02114, USA.

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http://dx.doi.org/10.1074/jbc.M109.098400DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2859537PMC
April 2010