Bosco Lab

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Publications

2017

ALS-linked FUS exerts a gain of toxic function involving aberrant p38 MAPK activation
Sama RR*, Fallini C*, Gatto R*, McKeon JE, Song Y, Rotunno MS, Penaranda S, Abdurakhmanov I, Landers JE, Morfini G, Brady ST, Bosco DA.
Sci Rep. 2017;7(1):115. (*co-first authors)

2015

Human C9ORF72 Hexanucleotide Expansion Reproduces RNA Foci and Dipeptide Repeat Proteins but Not Neurodegeneration in BAC Transgenic Mice
Peters, O, Cabrera, G, Tran, H, Gendron, T, McKeon, J, Metterville, J, Weiss, A, Nicholas, N, Salameh, J, Kim, J, Sun, H, Boylan, K, Dickson, D, Kennedy, Z, Lin, Z, Zhang, Y, Daughrity, L, Jung, C, Gao, F, Sapp, P, Horvitz, R, Bosco, DA, Brown, S, de Jong, P, Petrucelli, L, Mueller, C, Brown, RH, Jr
Neuron, 2015 Dec 2;88(5):902-9.


Structural basis for mutation-induced destabilization of profilin 1 in ALS

Boopathy, S*, Silvas, T*, Tischbein, M, Bilsel, O, Landers, JE, Shandilya, S, Schiffer C, Bosco DA.

Proc. Natl. Acad. Sci. USA. 2015 Jun 30;112(26):7984-9. (*co-first authors)

2014

Autophagy meets fused in sarcoma-positive stress granules.

Matus S, Bosco DA, Hetz C

Neurobiol. Aging, 2014 Dec;35(12):2832-5


A loss of FUS/TLS function leads to impaired cellular proliferation.
Ward CL, Boggio KJ, Johnson BN, Boyd JB, Douthwright S, Shaffer SA, Landers JE, Glicksman MA, Bosco DA.
Cell Death & Disease, Dec 11;5:e1572.


Identification of a Misfolded Region in Superoxide Dismutase 1 that is Exposed in Amyotrophic Lateral Sclerosis.

Rotunno MS, Auclair JR, Maniatis S, Shaffer SA, Agar J, Bosco DA.

J Biol Chem. 2014 Oct 10;289(41):28527-38.


Functions of FUS/TLS from DNA repair to Stress response: Implications of Amyotrophic Lateral Sclerosis.

Sama RR*, Ward CL*, Bosco DA. (* equal contribution)

ASN Neuro. 2014 Jun 1;6(4).


Artifacts to avoid while taking advantage of top-down mass spectrometry based detection of protein S-thiolation.

Auclair JR, Salisbury JP, Johnson JL, Petsko GA, Ringe D, Bosco DA, Agar NY, Santagata S, Durham HD, Agar JN.

Proteomics. 2014 May;14(10):1152-7.

2013

An emerging role for misfolded wild-type SOD1 in sporadic ALS pathogenesis.

Rotunno MS, Bosco DA.

Front Cell Neurosci. 2013 Dec 16;7:253.


Amyotrophic lateral sclerosis-linked FUS/TLS alters stress granule assembly and dynamics.

Baron DM, Kaushansky LJ, Ward CL, Sama RR, Chian RJ, Boggio KJ, Quaresma AJ, Nickerson JA, Bosco DA.

Mol Neurodegener. 2013 Aug 13;8(1):30.

Post-Translational Modification by Cysteine Protects Cu/Zn-Superoxide Dismutase from Oxidative Damage.

Auclair JR, Johnson JL, Liu Q, Salisbury JP, Rotunno MS, Petsko GA, Ringe D, Brown RH Jr, Bosco DA, Agar JN.

Biochemistry. 2013 Aug 26.

Inhibition of fast axonal transport by pathogenic SOD1 involves activation of p38 MAP kinase.

Morfini GA, Bosco DA, Brown H, Gatto R, Kaminska A, Song Y, Molla L, Baker L, Marangoni MN, Berth S, Tavassoli E, Bagnato C, Tiwari A, Hayward LJ, Pigino GF, Watterson DM, Huang CF, Banker G, Brown RH Jr, Brady ST.

PLoS One. 2013 Jun 12;8(6):e65235. 


FUS/TLS assembles into stress granules and is a prosurvival factor during hyperosmolar stress.

Sama RR, Ward CL, Kaushansky LJ, Lemay N, Ishigaki S, Urano F, Bosco DA.

J Cell Physiol. 2013 Nov;228(11):2222-31. 


Identification of human monoclonal antibodies specific for human SOD1 recognizing distinct epitopes and forms of SOD1.

Broering TJ, Wang H, Boatright NK, Wang Y, Baptista K, Shayan G, Garrity KA, Kayatekin C, Bosco DA, Matthews CR, Ambrosino DM, Xu Z, Babcock GJ.

PLoS One. 2013 Apr 17;8(4):e61210.

2012

Mutations in the profilin 1 gene cause familial amyotrophic lateral sclerosis.

Wu CH, Fallini C, Ticozzi N, Keagle PJ, Sapp PC, Piotrowska K, Lowe P, Koppers M, McKenna-Yasek D, Baron DM, Kost JE, Gonzalez-Perez P, Fox AD, Adams J, Taroni F, Tiloca C, Leclerc AL, Chafe SC, Mangroo D, Moore MJ, Zitzewitz JA, Xu ZS, van den Berg LH, Glass JD, Siciliano G, Cirulli ET, Goldstein DB, Salachas F, Meininger V, Rossoll W, Ratti A, Gellera C, Bosco DA, Bassell GJ, Silani V, Drory VE, Brown RH Jr, Landers JE.

Nature. 2012 Aug 23;488(7412):499-503. 


An over-oxidized form of superoxide dismutase found in sporadic amyotrophic lateral sclerosis with bulbar onset shares a toxic mechanism with mutant SOD1.

Guareschi S, Cova E, Cereda C, Ceroni M, Donetti E, Bosco DA, Trotti D, Pasinelli P.

Proc Natl Acad Sci U S A. 2012 Mar 27;109(13):5074-9.

2011

Anti-superoxide dismutase antibodies are associated with survival in patients with sporadic amyotrophic lateral sclerosis.

van Blitterswijk M, Gulati S, Smoot E, Jaffa M, Maher N, Hyman BT, Ivinson AJ, Scherzer CR, Schoenfeld DA, Cudkowicz ME, Brown RH Jr, Bosco DA.

Amyotroph Lateral Scler. 2011 Nov;12(6):430-8. 


Proteostasis and movement disorders: Parkinson's disease and amyotrophic lateral sclerosis.

Bosco DA, LaVoie MJ, Petsko GA, Ringe D.

Cold Spring Harb Perspect Biol. 2011 Oct 1;3(10):a007500.

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 Jr, Lindquist S, Ringe D, Petsko GA.

PLoS Biol. 2011 Apr;9(4):e1001052.

Mutational analysis reveals the FUS homolog TAF15 as a candidate gene for familial amyotrophic lateral sclerosis.

Ticozzi N, Vance C, Leclerc AL, Keagle P, Glass JD, McKenna-Yasek D, Sapp PC, Silani V, Bosco DA, Shaw CE, Brown RH Jr, Landers JE.

Am J Med Genet B Neuropsychiatr Genet. 2011 Apr;156B(3):285-90.

2010

Wild-type and mutant SOD1 share an aberrant conformation and a common pathogenic pathway in ALS.

Bosco DA, Morfini G, Karabacak NM, Song Y, Gros-Louis F, Pasinelli P, Goolsby H, Fontaine BA, Lemay N, McKenna-Yasek D, Frosch MP, Agar JN, Julien JP, Brady ST, Brown RH Jr.

Nat Neurosci. 2010 Nov;13(11):1396-403.

Genetic determinants of amyotrophic lateral sclerosis as therapeutic targets.

Bosco DA, Landers JE.

CNS Neurol Disord Drug Targets. 2010 Dec;9(6):779-90.

Dissecting the microscopic steps of the cyclophilin A enzymatic cycle on the biological HIV-1 capsid substrate by NMR.

Bosco DA, Eisenmesser EZ, Clarkson MW, Wolf-Watz M, Labeikovsky W, Millet O, Kern D.

J Mol Biol. 2010 Nov 12;403(5):723-38.

Mutant FUS proteins that cause amyotrophic lateral sclerosis incorporate into stress granules.

Bosco DA, Lemay N, Ko HK, Zhou H, Burke C, Kwiatkowski TJ Jr, Sapp P, McKenna-Yasek D, Brown RH Jr, Hayward LJ.

Hum Mol Genet. 2010 Nov 1;19(21):4160-75.

Paraoxonase gene mutations in amyotrophic lateral sclerosis.

Ticozzi N, LeClerc AL, Keagle PJ, Glass JD, Wills AM, van Blitterswijk M, Bosco DA, Rodriguez-Leyva I, Gellera C, Ratti A, Taroni F, McKenna-Yasek D, Sapp PC, Silani V, Furlong CE, Brown RH Jr, Landers JE.

Ann Neurol. 2010 Jul;68(1):102-7.

2009

Axonal transport defects in neurodegenerative diseases.

Morfini GA, Burns M, Binder LI, Kanaan NM, LaPointe N, Bosco DA, Brown RH Jr, Brown H, Tiwari A, Hayward L, Edgar J, Nave KA, Garberrn J, Atagi Y, Song Y, Pigino G, Brady ST.

J Neurosci. 2009 Oct 14;29(41):12776-86. doi: 10.1523/JNEUROSCI.3463-09.2009.


Localized thermodynamic coupling between hydrogen bonding and microenvironment polarity substantially stabilizes proteins.

Gao J, Bosco DA, Powers ET, Kelly JW.

Nat Struct Mol Biol. 2009 Jul;16(7):684-90.

Mutations in the FUS/TLS gene on chromosome 16 cause familial amyotrophic lateral sclerosis.

Kwiatkowski TJ Jr, Bosco DA, Leclerc AL, Tamrazian E, Vanderburg CR, Russ C, Davis A, Gilchrist J, Kasarskis EJ, Munsat T, Valdmanis P, Rouleau GA, Hosler BA, Cortelli P, de Jong PJ, Yoshinaga Y, Haines JL, Pericak-Vance MA, Yan J, Ticozzi N, Siddique T, McKenna-Yasek D, Sapp PC, Horvitz HR, Landers JE, Brown RH Jr.

Science. 2009 Feb 27;323(5918):1205-8.

2000-08

Structure and dynamics of pin1 during catalysis by NMR.

Labeikovsky W, Eisenmesser EZ, Bosco DA, Kern D.

J Mol Biol. 2007 Apr 13;367(5):1370-81.

Small molecule oxidation products trigger disease-associated protein misfolding.

Bieschke J, Zhang Q, Bosco DA, Lerner RA, Powers ET, Wentworth P Jr, Kelly JW.

Acc Chem Res. 2006 Sep;39(9):611-9.

Elevated levels of oxidized cholesterol metabolites in Lewy body disease brains accelerate alpha-synuclein fibrilization.

Bosco DA, Fowler DM, Zhang Q, Nieva J, Powers ET, Wentworth P Jr, Lerner RA, Kelly JW.

Nat Chem Biol. 2006 May;2(5):249-53. Epub 2006 Mar 26. Erratum in: Nat Chem Biol. 2006 Jun;2(6):346.


Intrinsic dynamics of an enzyme underlies catalysis.

Eisenmesser EZ, Millet O, Labeikovsky W, Korzhnev DM, Wolf-Watz M, Bosco DA, Skalicky JJ, Kay LE, Kern D.

Nature. 2005 Nov 3;438(7064):117-21.


Catalysis and binding of cyclophilin A with different HIV-1 capsid constructs.

Bosco DA, Kern D.

Biochemistry. 2004 May 25;43(20):6110-9.


Catalysis of cis/trans isomerization in native HIV-1 capsid by human cyclophilin A.

Bosco DA, Eisenmesser EZ, Pochapsky S, Sundquist WI, Kern D.

Proc Natl Acad Sci U S A. 2002 Apr 16;99(8):5247-52

Enzyme dynamics during catalysis.

Eisenmesser EZ, Bosco DA, Akke M, Kern D.

Science. 2002 Feb 22;295(5559):1520-3.