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Items: 1 to 20 of 44


Lysyl-tRNA synthetase is a target for mutant SOD1 toxicity in mitochondria.

Kawamata H, Magrané J, Kunst C, King MP, Manfredi G.

J Biol Chem. 2008 Oct 17;283(42):28321-8. doi: 10.1074/jbc.M805599200. Epub 2008 Aug 20.


Different regulation of wild-type and mutant Cu,Zn superoxide dismutase localization in mammalian mitochondria.

Kawamata H, Manfredi G.

Hum Mol Genet. 2008 Nov 1;17(21):3303-17. doi: 10.1093/hmg/ddn226. Epub 2008 Aug 13.


Mutant SOD1 in neuronal mitochondria causes toxicity and mitochondrial dynamics abnormalities.

Magrané J, Hervias I, Henning MS, Damiano M, Kawamata H, Manfredi G.

Hum Mol Genet. 2009 Dec 1;18(23):4552-64. doi: 10.1093/hmg/ddp421. Epub 2009 Sep 24.


Mutations in SOD1 associated with amyotrophic lateral sclerosis cause novel protein interactions.

Kunst CB, Mezey E, Brownstein MJ, Patterson D.

Nat Genet. 1997 Jan;15(1):91-4.


Glutaredoxin 2 prevents aggregation of mutant SOD1 in mitochondria and abolishes its toxicity.

Ferri A, Fiorenzo P, Nencini M, Cozzolino M, Pesaresi MG, Valle C, Sepe S, Moreno S, Carrì MT.

Hum Mol Genet. 2010 Nov 15;19(22):4529-42. doi: 10.1093/hmg/ddq383. Epub 2010 Sep 9.


ALS-linked mutant superoxide dismutase 1 (SOD1) alters mitochondrial protein composition and decreases protein import.

Li Q, Vande Velde C, Israelson A, Xie J, Bailey AO, Dong MQ, Chun SJ, Roy T, Winer L, Yates JR, Capaldi RA, Cleveland DW, Miller TM.

Proc Natl Acad Sci U S A. 2010 Dec 7;107(49):21146-51. doi: 10.1073/pnas.1014862107. Epub 2010 Nov 15.


CuZn superoxide dismutase (SOD1) accumulates in vacuolated mitochondria in transgenic mice expressing amyotrophic lateral sclerosis-linked SOD1 mutations.

Jaarsma D, Rognoni F, van Duijn W, Verspaget HW, Haasdijk ED, Holstege JC.

Acta Neuropathol. 2001 Oct;102(4):293-305.


Mutated human SOD1 causes dysfunction of oxidative phosphorylation in mitochondria of transgenic mice.

Mattiazzi M, D'Aurelio M, Gajewski CD, Martushova K, Kiaei M, Beal MF, Manfredi G.

J Biol Chem. 2002 Aug 16;277(33):29626-33. Epub 2002 Jun 5.


Import, maturation, and function of SOD1 and its copper chaperone CCS in the mitochondrial intermembrane space.

Kawamata H, Manfredi G.

Antioxid Redox Signal. 2010 Nov 1;13(9):1375-84. doi: 10.1089/ars.2010.3212. Review.


Misfolded SOD1 associated with motor neuron mitochondria alters mitochondrial shape and distribution prior to clinical onset.

Vande Velde C, McDonald KK, Boukhedimi Y, McAlonis-Downes M, Lobsiger CS, Bel Hadj S, Zandona A, Julien JP, Shah SB, Cleveland DW.

PLoS One. 2011;6(7):e22031. doi: 10.1371/journal.pone.0022031. Epub 2011 Jul 11.


Mitochondrial dysfunction in amyotrophic lateral sclerosis.

Shi P, Gal J, Kwinter DM, Liu X, Zhu H.

Biochim Biophys Acta. 2010 Jan;1802(1):45-51. doi: 10.1016/j.bbadis.2009.08.012. Epub 2009 Aug 26. Review.


ALS-linked mutant SOD1 damages mitochondria by promoting conformational changes in Bcl-2.

Pedrini S, Sau D, Guareschi S, Bogush M, Brown RH Jr, Naniche N, Kia A, Trotti D, Pasinelli P.

Hum Mol Genet. 2010 Aug 1;19(15):2974-86. doi: 10.1093/hmg/ddq202. Epub 2010 May 11.


Expression of a Cu,Zn superoxide dismutase typical for familial amyotrophic lateral sclerosis increases the vulnerability of neuroblastoma cells to infectious injury.

Goos M, Zech WD, Jaiswal MK, Balakrishnan S, Ebert S, Mitchell T, Carrì MT, Keller BU, Nau R.

BMC Infect Dis. 2007 Nov 12;7:131.


Defective mitochondrial dynamics is an early event in skeletal muscle of an amyotrophic lateral sclerosis mouse model.

Luo G, Yi J, Ma C, Xiao Y, Yi F, Yu T, Zhou J.

PLoS One. 2013 Dec 6;8(12):e82112. doi: 10.1371/journal.pone.0082112. eCollection 2013.


G37R SOD1 mutant alters mitochondrial complex I activity, Ca(2+) uptake and ATP production.

Coussee E, De Smet P, Bogaert E, Elens I, Van Damme P, Willems P, Koopman W, Van Den Bosch L, Callewaert G.

Cell Calcium. 2011 Apr;49(4):217-25. doi: 10.1016/j.ceca.2011.02.004. Epub 2011 Mar 8.


Oxidative stress, mutant SOD1, and neurofilament pathology in transgenic mouse models of human motor neuron disease.

Tu PH, Gurney ME, Julien JP, Lee VM, Trojanowski JQ.

Lab Invest. 1997 Apr;76(4):441-56. Review. Erratum in: Lab Invest 1997 Jun;76(6):followi.


Transgenics, toxicity and therapeutics in rodent models of mutant SOD1-mediated familial ALS.

Turner BJ, Talbot K.

Prog Neurobiol. 2008 May;85(1):94-134. doi: 10.1016/j.pneurobio.2008.01.001. Epub 2008 Jan 16. Review.


Impairment of mitochondrial anti-oxidant defence in SOD1-related motor neuron injury and amelioration by ebselen.

Wood-Allum CA, Barber SC, Kirby J, Heath P, Holden H, Mead R, Higginbottom A, Allen S, Beaujeux T, Alexson SE, Ince PG, Shaw PJ.

Brain. 2006 Jul;129(Pt 7):1693-709. Epub 2006 May 15.


Mutation of SOD1 in ALS: a gain of a loss of function.

Sau D, De Biasi S, Vitellaro-Zuccarello L, Riso P, Guarnieri S, Porrini M, Simeoni S, Crippa V, Onesto E, Palazzolo I, Rusmini P, Bolzoni E, Bendotti C, Poletti A.

Hum Mol Genet. 2007 Jul 1;16(13):1604-18. Epub 2007 May 15.


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