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Items: 1 to 20 of 28


Pathogenesis of FUS-associated ALS and FTD: insights from rodent models.

Nolan M, Talbot K, Ansorge O.

Acta Neuropathol Commun. 2016 Sep 6;4(1):99. doi: 10.1186/s40478-016-0358-8. Review.


The Enemy within: Innate Surveillance-Mediated Cell Death, the Common Mechanism of Neurodegenerative Disease.

Richards RI, Robertson SA, O'Keefe LV, Fornarino D, Scott A, Lardelli M, Baune BT.

Front Neurosci. 2016 May 10;10:193. doi: 10.3389/fnins.2016.00193. eCollection 2016.


The role of CHMP2BIntron5 in autophagy and frontotemporal dementia.

Krasniak CS, Ahmad ST.

Brain Res. 2016 Oct 15;1649(Pt B):151-157. doi: 10.1016/j.brainres.2016.02.051. Epub 2016 Mar 10. Review.


Haploinsufficiency of cathepsin D leads to lysosomal dysfunction and promotes cell-to-cell transmission of α-synuclein aggregates.

Bae EJ, Yang NY, Lee C, Kim S, Lee HJ, Lee SJ.

Cell Death Dis. 2015 Oct 8;6:e1901. doi: 10.1038/cddis.2015.283.


Personality disturbances in amyotrophic lateral sclerosis: a case study demonstrating changes in personality without cognitive deficits.

Waldron EJ, Barrash J, Swenson A, Tranel D.

J Int Neuropsychol Soc. 2014 Aug;20(7):764-71. doi: 10.1017/S1355617714000459. Epub 2014 May 23.


Calcium ions promote superoxide dismutase 1 (SOD1) aggregation into non-fibrillar amyloid: a link to toxic effects of calcium overload in amyotrophic lateral sclerosis (ALS)?

Leal SS, Cardoso I, Valentine JS, Gomes CM.

J Biol Chem. 2013 Aug 30;288(35):25219-28. doi: 10.1074/jbc.M113.470740. Epub 2013 Jul 16.


Need an ESCRT for autophagosomal maturation?

Manil-Segalén M, Lefebvre C, Culetto E, Legouis R.

Commun Integr Biol. 2012 Nov 1;5(6):566-71. doi: 10.4161/cib.21522.


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. doi: 10.1073/pnas.1115402109. Epub 2012 Mar 13.


C9orf72 repeat expansions in patients with ALS and FTD.

Rademakers R.

Lancet Neurol. 2012 Apr;11(4):297-8. doi: 10.1016/S1474-4422(12)70046-7. Epub 2012 Mar 9. No abstract available.


Valosin-containing protein and neurofibromin interact to regulate dendritic spine density.

Wang HF, Shih YT, Chen CY, Chao HW, Lee MJ, Hsueh YP.

J Clin Invest. 2011 Dec;121(12):4820-37. doi: 10.1172/JCI45677. Epub 2011 Nov 21.


Aging--RNA in development and disease.

Cookson MR.

Wiley Interdiscip Rev RNA. 2012 Jan-Feb;3(1):133-43. doi: 10.1002/wrna.109. Epub 2011 Sep 6. Review.


Inflammation in the early stages of neurodegenerative pathology.

Khandelwal PJ, Herman AM, Moussa CE.

J Neuroimmunol. 2011 Sep 15;238(1-2):1-11. doi: 10.1016/j.jneuroim.2011.07.002. Epub 2011 Aug 5. Review.


The Relationship between Parkin and Protein Aggregation in Neurodegenerative Diseases.

Khandelwal PJ, Moussa CE.

Front Psychiatry. 2010 Jun 3;1:15. doi: 10.3389/fpsyt.2010.00015. eCollection 2010.


β-amyloid triggers ALS-associated TDP-43 pathology in AD models.

Herman AM, Khandelwal PJ, Stanczyk BB, Rebeck GW, Moussa CE.

Brain Res. 2011 Apr 22;1386:191-9. doi: 10.1016/j.brainres.2011.02.052. Epub 2011 Mar 2.


Dysregulation of the ALS-associated gene TDP-43 leads to neuronal death and degeneration in mice.

Igaz LM, Kwong LK, Lee EB, Chen-Plotkin A, Swanson E, Unger T, Malunda J, Xu Y, Winton MJ, Trojanowski JQ, Lee VM.

J Clin Invest. 2011 Feb;121(2):726-38. doi: 10.1172/JCI44867. Epub 2011 Jan 4.


ALS-associated fused in sarcoma (FUS) mutations disrupt Transportin-mediated nuclear import.

Dormann D, Rodde R, Edbauer D, Bentmann E, Fischer I, Hruscha A, Than ME, Mackenzie IR, Capell A, Schmid B, Neumann M, Haass C.

EMBO J. 2010 Aug 18;29(16):2841-57. doi: 10.1038/emboj.2010.143. Epub 2010 Jul 6.


TAR DNA-binding protein 43 in neurodegenerative disease.

Chen-Plotkin AS, Lee VM, Trojanowski JQ.

Nat Rev Neurol. 2010 Apr;6(4):211-20. doi: 10.1038/nrneurol.2010.18. Epub 2010 Mar 16. Review.


Structural insights into TDP-43 in nucleic-acid binding and domain interactions.

Kuo PH, Doudeva LG, Wang YT, Shen CK, Yuan HS.

Nucleic Acids Res. 2009 Apr;37(6):1799-808. doi: 10.1093/nar/gkp013. Epub 2009 Jan 27.


Pedigree with frontotemporal lobar degeneration--motor neuron disease and Tar DNA binding protein-43 positive neuropathology: genetic linkage to chromosome 9.

Luty AA, Kwok JB, Thompson EM, Blumbergs P, Brooks WS, Loy CT, Dobson-Stone C, Panegyres PK, Hecker J, Nicholson GA, Halliday GM, Schofield PR.

BMC Neurol. 2008 Aug 29;8:32. doi: 10.1186/1471-2377-8-32.


Mucolipidosis type IV: the importance of functional lysosomes for efficient autophagy.

Vergarajauregui S, Puertollano R.

Autophagy. 2008 Aug;4(6):832-4. Epub 2008 Jul 8.

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