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Results: 1 to 20 of 102

1.

Misfolded mutant SOD1 directly inhibits VDAC1 conductance in a mouse model of inherited ALS.

Israelson A, Arbel N, Da Cruz S, Ilieva H, Yamanaka K, Shoshan-Barmatz V, Cleveland DW.

Neuron. 2010 Aug 26;67(4):575-87. doi: 10.1016/j.neuron.2010.07.019.

PMID:
20797535
[PubMed - indexed for MEDLINE]
Free PMC Article
2.

Enhancing mitochondrial calcium buffering capacity reduces aggregation of misfolded SOD1 and motor neuron cell death without extending survival in mouse models of inherited amyotrophic lateral sclerosis.

Parone PA, Da Cruz S, Han JS, McAlonis-Downes M, Vetto AP, Lee SK, Tseng E, Cleveland DW.

J Neurosci. 2013 Mar 13;33(11):4657-71. doi: 10.1523/JNEUROSCI.1119-12.2013.

PMID:
23486940
[PubMed - indexed for MEDLINE]
Free PMC Article
3.

Mitochondrial damage revealed by immunoselection for ALS-linked misfolded SOD1.

Pickles S, Destroismaisons L, Peyrard SL, Cadot S, Rouleau GA, Brown RH Jr, Julien JP, Arbour N, Vande Velde C.

Hum Mol Genet. 2013 Oct 1;22(19):3947-59. doi: 10.1093/hmg/ddt249. Epub 2013 Jun 4.

PMID:
23736301
[PubMed - indexed for MEDLINE]
4.

Selective association of misfolded ALS-linked mutant SOD1 with the cytoplasmic face of mitochondria.

Vande Velde C, Miller TM, Cashman NR, Cleveland DW.

Proc Natl Acad Sci U S A. 2008 Mar 11;105(10):4022-7. doi: 10.1073/pnas.0712209105. Epub 2008 Feb 22.

PMID:
18296640
[PubMed - indexed for MEDLINE]
Free PMC Article
5.

The mitochondrial permeability transition pore in motor neurons: involvement in the pathobiology of ALS mice.

Martin LJ, Gertz B, Pan Y, Price AC, Molkentin JD, Chang Q.

Exp Neurol. 2009 Aug;218(2):333-46. doi: 10.1016/j.expneurol.2009.02.015. Epub 2009 Mar 9.

PMID:
19272377
[PubMed - indexed for MEDLINE]
Free PMC Article
6.

Proteins that bind to misfolded mutant superoxide dismutase-1 in spinal cords from transgenic amyotrophic lateral sclerosis (ALS) model mice.

Zetterström P, Graffmo KS, Andersen PM, Brännström T, Marklund SL.

J Biol Chem. 2011 Jun 10;286(23):20130-6. doi: 10.1074/jbc.M111.218842. Epub 2011 Apr 14.

PMID:
21493711
[PubMed - indexed for MEDLINE]
Free PMC Article
7.

Nuclear localization of human SOD1 and mutant SOD1-specific disruption of survival motor neuron protein complex in transgenic amyotrophic lateral sclerosis mice.

Gertz B, Wong M, Martin LJ.

J Neuropathol Exp Neurol. 2012 Feb;71(2):162-77. doi: 10.1097/NEN.0b013e318244b635.

PMID:
22249462
[PubMed - indexed for MEDLINE]
Free PMC Article
8.

Copper and zinc metallation status of copper-zinc superoxide dismutase from amyotrophic lateral sclerosis transgenic mice.

Lelie HL, Liba A, Bourassa MW, Chattopadhyay M, Chan PK, Gralla EB, Miller LM, Borchelt DR, Valentine JS, Whitelegge JP.

J Biol Chem. 2011 Jan 28;286(4):2795-806. doi: 10.1074/jbc.M110.186999. Epub 2010 Nov 10.

PMID:
21068388
[PubMed - indexed for MEDLINE]
Free PMC Article
9.

In vivo pathogenic role of mutant SOD1 localized in the mitochondrial intermembrane space.

Igoudjil A, Magrané J, Fischer LR, Kim HJ, Hervias I, Dumont M, Cortez C, Glass JD, Starkov AA, Manfredi G.

J Neurosci. 2011 Nov 2;31(44):15826-37. doi: 10.1523/JNEUROSCI.1965-11.2011.

PMID:
22049426
[PubMed - indexed for MEDLINE]
Free PMC Article
10.

Survival motor neuron deficiency enhances progression in an amyotrophic lateral sclerosis mouse model.

Turner BJ, Parkinson NJ, Davies KE, Talbot K.

Neurobiol Dis. 2009 Jun;34(3):511-7. doi: 10.1016/j.nbd.2009.03.005. Epub 2009 Mar 28.

PMID:
19332122
[PubMed - indexed for MEDLINE]
11.

Heat shock factor 1 over-expression protects against exposure of hydrophobic residues on mutant SOD1 and early mortality in a mouse model of amyotrophic lateral sclerosis.

Lin PY, Simon SM, Koh WK, Folorunso O, Umbaugh CS, Pierce A.

Mol Neurodegener. 2013 Nov 21;8:43. doi: 10.1186/1750-1326-8-43.

PMID:
24256636
[PubMed - indexed for MEDLINE]
Free PMC Article
12.

Aberrant localization of FUS and TDP43 is associated with misfolding of SOD1 in amyotrophic lateral sclerosis.

Pokrishevsky E, Grad LI, Yousefi M, Wang J, Mackenzie IR, Cashman NR.

PLoS One. 2012;7(4):e35050. doi: 10.1371/journal.pone.0035050. Epub 2012 Apr 6.

PMID:
22493728
[PubMed - indexed for MEDLINE]
Free PMC Article
13.

The small heat shock protein B8 (HspB8) promotes autophagic removal of misfolded proteins involved in amyotrophic lateral sclerosis (ALS).

Crippa V, Sau D, Rusmini P, Boncoraglio A, Onesto E, Bolzoni E, Galbiati M, Fontana E, Marino M, Carra S, Bendotti C, De Biasi S, Poletti A.

Hum Mol Genet. 2010 Sep 1;19(17):3440-56. doi: 10.1093/hmg/ddq257. Epub 2010 Jun 22.

PMID:
20570967
[PubMed - indexed for MEDLINE]
Free Article
14.

Mutant SOD1G93A triggers mitochondrial fragmentation in spinal cord motor neurons: neuroprotection by SIRT3 and PGC-1α.

Song W, Song Y, Kincaid B, Bossy B, Bossy-Wetzel E.

Neurobiol Dis. 2013 Mar;51:72-81. doi: 10.1016/j.nbd.2012.07.004. Epub 2012 Jul 20.

PMID:
22819776
[PubMed - indexed for MEDLINE]
Free PMC Article
15.

Targeting of monomer/misfolded SOD1 as a therapeutic strategy for amyotrophic lateral sclerosis.

Liu HN, Tjostheim S, Dasilva K, Taylor D, Zhao B, Rakhit R, Brown M, Chakrabartty A, McLaurin J, Robertson J.

J Neurosci. 2012 Jun 27;32(26):8791-9. doi: 10.1523/JNEUROSCI.5053-11.2012.

PMID:
22745481
[PubMed - indexed for MEDLINE]
Free Article
16.

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.

PMID:
21779368
[PubMed - indexed for MEDLINE]
Free PMC Article
17.

Molecular chaperone mediated late-stage neuroprotection in the SOD1(G93A) mouse model of amyotrophic lateral sclerosis.

Novoselov SS, Mustill WJ, Gray AL, Dick JR, Kanuga N, Kalmar B, Greensmith L, Cheetham ME.

PLoS One. 2013 Aug 30;8(8):e73944. doi: 10.1371/journal.pone.0073944. eCollection 2013.

PMID:
24023695
[PubMed - indexed for MEDLINE]
Free PMC Article
18.

Modulation of astrocytic mitochondrial function by dichloroacetate improves survival and motor performance in inherited amyotrophic lateral sclerosis.

Miquel E, Cassina A, Martínez-Palma L, Bolatto C, Trías E, Gandelman M, Radi R, Barbeito L, Cassina P.

PLoS One. 2012;7(4):e34776. doi: 10.1371/journal.pone.0034776. Epub 2012 Apr 3.

PMID:
22509356
[PubMed - indexed for MEDLINE]
Free PMC Article
19.

Human Cu/Zn superoxide dismutase (SOD1) overexpression in mice causes mitochondrial vacuolization, axonal degeneration, and premature motoneuron death and accelerates motoneuron disease in mice expressing a familial amyotrophic lateral sclerosis mutant SOD1.

Jaarsma D, Haasdijk ED, Grashorn JA, Hawkins R, van Duijn W, Verspaget HW, London J, Holstege JC.

Neurobiol Dis. 2000 Dec;7(6 Pt B):623-43.

PMID:
11114261
[PubMed - indexed for MEDLINE]
20.

Mutant SOD1 from spinal cord of G93A rats is destabilized and binds to inner mitochondrial membrane.

Ahtoniemi T, Jaronen M, Keksa-Goldsteine V, Goldsteins G, Koistinaho J.

Neurobiol Dis. 2008 Dec;32(3):479-85. doi: 10.1016/j.nbd.2008.08.010. Epub 2008 Sep 9.

PMID:
18817872
[PubMed - indexed for MEDLINE]

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