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    Last Name

    Elisabet Mandon PhD

    TitleAssociate Professor
    InstitutionUniversity of Massachusetts Medical School
    DepartmentBiochemistry and Molecular Pharmacology
    AddressUniversity of Massachusetts Medical School
    364 Plantation Street, LRB-970
    Worcester MA 01605
      Other Positions
      InstitutionUMMS - School of Medicine
      DepartmentBiochemistry and Molecular Pharmacology

      InstitutionUMMS - Graduate School of Biomedical Sciences
      DepartmentBiochemistry and Molecular Pharmacology

        Lab web-page:

        Post Docs

        A postdoctoral research position is available  to investigate Amyotrophic LateralSclerosis (ALS) mitochondrial dysfunction. ALS causes motor neurondegeneration, then paralysis and death. An early target in ALS is themitochondrion, whose dysfunction enhances ALS progression. The most commonALS-causing mutations are found in the copper-zinc superoxide dismutase (SOD1)gene. Most wild type (WT) SOD1 protein (SOD1WT) resides in the cytosol and only1% is targeted to the inner mitochondrial space (IMS). In contrast, mutant SOD1protein (SOD1mut) accumulates in the mitochondrial outer membrane and in thematrix, and subsequently impairs mitochondrial function by a mechanism that ispoorly understood. Abnormal SOD1mut accumulation leads to mitochondrialdysfunction found in the early stage of presymptomatic ALS mice, before theonset of motor neuron degeneration. Thus, it is critical to determine the firstevent induced by SOD1mut and devise a strategy to stop or delay it beforemitochondrial damage becomes irreversible. The objective of future studies willbe to determine which step/steps are affected by the SOD1 mutation byidentifying the sequence of events involved in SOD1mut and SOD1WT mitochondrialtargeting, import and IMS-sorting. This project will provide important basicinformation on the mechanisms underlying mitochondrial dysfunction in ALS andwill identify possible targets for future therapies.

                     Applicants must have (or expect toobtain shortly) a Ph.D. in Biochemistry, Cell Biology, Molecular Biology or arelated field and be strongly motivated to conduct research in an excitingresearch environment. 


        selected publications
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        1. Mandon EC, Trueman SF, Gilmore R. Protein translocation across the rough endoplasmic reticulum. Cold Spring Harb Perspect Biol. 2013 Feb 01; 5(2).
          View in: PubMed
        2. Trueman SF, Mandon EC, Gilmore R. A gating motif in the translocation channel sets the hydrophobicity threshold for signal sequence function. J Cell Biol. 2012 Dec 10; 199(6):907-18.
          View in: PubMed
        3. Gilmore R, Mandon EC. Understanding integration of a-helical membrane proteins: the next steps. Trends Biochem Sci. 2012 Aug; 37(8):303-8.
          View in: PubMed
        4. Trueman SF, Mandon EC, Gilmore R. Translocation channel gating kinetics balances protein translocation efficiency with signal sequence recognition fidelity. Mol Biol Cell. 2011 Sep; 22(17):2983-93.
          View in: PubMed
        5. Becker T, Bhushan S, Jarasch A, Armache JP, Funes S, Jossinet F, Gumbart J, Mielke T, Berninghausen O, Schulten K, Westhof E, Gilmore R, Mandon EC, Beckmann R. Structure of monomeric yeast and mammalian Sec61 complexes interacting with the translating ribosome. Science. 2009 Dec 4; 326(5958):1369-73.
          View in: PubMed
        6. Mandon EC, Trueman SF, Gilmore R. Translocation of proteins through the Sec61 and SecYEG channels. Curr Opin Cell Biol. 2009 Aug; 21(4):501-7.
          View in: PubMed
        7. Jiang Y, Cheng Z, Mandon EC, Gilmore R. An interaction between the SRP receptor and the translocon is critical during cotranslational protein translocation. J Cell Biol. 2008 Mar 24; 180(6):1149-61.
          View in: PubMed
        8. Mandon EC, Gilmore R. The tail end of membrane insertion. Cell. 2007 Mar 23; 128(6):1031-2.
          View in: PubMed
        9. Cheng Z, Jiang Y, Mandon EC, Gilmore R. Identification of cytoplasmic residues of Sec61p involved in ribosome binding and cotranslational translocation. J Cell Biol. 2005 Jan 3; 168(1):67-77.
          View in: PubMed
        10. Mandon EC, Gilmore R. GTPase twins in the SRP family. Nat Struct Mol Biol. 2004 Feb; 11(2):115-6.
          View in: PubMed
        11. Mandon EC, Jiang Y, Gilmore R. Dual recognition of the ribosome and the signal recognition particle by the SRP receptor during protein targeting to the endoplasmic reticulum. J Cell Biol. 2003 Aug 18; 162(4):575-85.
          View in: PubMed
        12. Kelleher DJ, Karaoglu D, Mandon EC, Gilmore R. Oligosaccharyltransferase isoforms that contain different catalytic STT3 subunits have distinct enzymatic properties. Mol Cell. 2003 Jul; 12(1):101-11.
          View in: PubMed
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