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Items: 1 to 20 of 170

1.

Motor neuron disease, TDP-43 pathology, and memory deficits in mice expressing ALS-FTD-linked UBQLN2 mutations.

Le NT, Chang L, Kovlyagina I, Georgiou P, Safren N, Braunstein KE, Kvarta MD, Van Dyke AM, LeGates TA, Philips T, Morrison BM, Thompson SM, Puche AC, Gould TD, Rothstein JD, Wong PC, Monteiro MJ.

Proc Natl Acad Sci U S A. 2016 Nov 22;113(47):E7580-E7589. Epub 2016 Nov 9.

2.

Viral expression of ALS-linked ubiquilin-2 mutants causes inclusion pathology and behavioral deficits in mice.

Ceballos-Diaz C, Rosario AM, Park HJ, Chakrabarty P, Sacino A, Cruz PE, Siemienski Z, Lara N, Moran C, Ravelo N, Golde TE, McFarland NR.

Mol Neurodegener. 2015 Jul 8;10:25. doi: 10.1186/s13024-015-0026-7.

3.

Evidence of a link between ubiquilin 2 and optineurin in amyotrophic lateral sclerosis.

Osaka M, Ito D, Yagi T, Nihei Y, Suzuki N.

Hum Mol Genet. 2015 Mar 15;24(6):1617-29. doi: 10.1093/hmg/ddu575. Epub 2014 Nov 14.

PMID:
25398946
4.

TDP-43 expression in mouse models of amyotrophic lateral sclerosis and spinal muscular atrophy.

Turner BJ, Bäumer D, Parkinson NJ, Scaber J, Ansorge O, Talbot K.

BMC Neurosci. 2008 Oct 28;9:104. doi: 10.1186/1471-2202-9-104.

5.

Novel UBQLN2 mutations linked to amyotrophic lateral sclerosis and atypical hereditary spastic paraplegia phenotype through defective HSP70-mediated proteolysis.

Teyssou E, Chartier L, Amador MD, Lam R, Lautrette G, Nicol M, Machat S, Da Barroca S, Moigneu C, Mairey M, Larmonier T, Saker S, Dussert C, Forlani S, Fontaine B, Seilhean D, Bohl D, Boillée S, Meininger V, Couratier P, Salachas F, Stevanin G, Millecamps S.

Neurobiol Aging. 2017 Oct;58:239.e11-239.e20. doi: 10.1016/j.neurobiolaging.2017.06.018. Epub 2017 Jun 24.

PMID:
28716533
6.

UBQLN2/ubiquilin 2 mutation and pathology in familial amyotrophic lateral sclerosis.

Williams KL, Warraich ST, Yang S, Solski JA, Fernando R, Rouleau GA, Nicholson GA, Blair IP.

Neurobiol Aging. 2012 Oct;33(10):2527.e3-10. doi: 10.1016/j.neurobiolaging.2012.05.008. Epub 2012 Jun 19.

PMID:
22717235
7.

Mutation-dependent aggregation and toxicity in a Drosophila model for UBQLN2-associated ALS.

Kim SH, Stiles SG, Feichtmeier JM, Ramesh N, Zhan L, Scalf MA, Smith LM, Pandey UB, Tibbetts RS.

Hum Mol Genet. 2018 Jan 15;27(2):322-337. doi: 10.1093/hmg/ddx403.

8.

Clinical and neuropathologic heterogeneity of c9FTD/ALS associated with hexanucleotide repeat expansion in C9ORF72.

Murray ME, DeJesus-Hernandez M, Rutherford NJ, Baker M, Duara R, Graff-Radford NR, Wszolek ZK, Ferman TJ, Josephs KA, Boylan KB, Rademakers R, Dickson DW.

Acta Neuropathol. 2011 Dec;122(6):673-90. doi: 10.1007/s00401-011-0907-y. Epub 2011 Nov 15.

9.

Lack of TDP-43 abnormalities in mutant SOD1 transgenic mice shows disparity with ALS.

Robertson J, Sanelli T, Xiao S, Yang W, Horne P, Hammond R, Pioro EP, Strong MJ.

Neurosci Lett. 2007 Jun 13;420(2):128-32. Epub 2007 Apr 8.

PMID:
17543992
10.

Functional recovery in new mouse models of ALS/FTLD after clearance of pathological cytoplasmic TDP-43.

Walker AK, Spiller KJ, Ge G, Zheng A, Xu Y, Zhou M, Tripathy K, Kwong LK, Trojanowski JQ, Lee VM.

Acta Neuropathol. 2015 Nov;130(5):643-60. doi: 10.1007/s00401-015-1460-x. Epub 2015 Jul 22.

11.

Neuronal Expression of UBQLN2P497H Exacerbates TDP-43 Pathology in TDP-43G348C Mice through Interaction with Ubiquitin.

Picher-Martel V, Renaud L, Bareil C, Julien JP.

Mol Neurobiol. 2019 Jul;56(7):4680-4696. doi: 10.1007/s12035-018-1411-3. Epub 2018 Oct 30.

12.

Comparative interactomics analysis of different ALS-associated proteins identifies converging molecular pathways.

Blokhuis AM, Koppers M, Groen EJN, van den Heuvel DMA, Dini Modigliani S, Anink JJ, Fumoto K, van Diggelen F, Snelting A, Sodaar P, Verheijen BM, Demmers JAA, Veldink JH, Aronica E, Bozzoni I, den Hertog J, van den Berg LH, Pasterkamp RJ.

Acta Neuropathol. 2016 Aug;132(2):175-196. doi: 10.1007/s00401-016-1575-8. Epub 2016 May 10.

13.

Disturbance of proteasomal and autophagic protein degradation pathways by amyotrophic lateral sclerosis-linked mutations in ubiquilin 2.

Osaka M, Ito D, Suzuki N.

Biochem Biophys Res Commun. 2016 Apr 1;472(2):324-31. doi: 10.1016/j.bbrc.2016.02.107. Epub 2016 Mar 2.

PMID:
26944018
14.

A transgenic mouse expressing CHMP2Bintron5 mutant in neurons develops histological and behavioural features of amyotrophic lateral sclerosis and frontotemporal dementia.

Vernay A, Therreau L, Blot B, Risson V, Dirrig-Grosch S, Waegaert R, Lequeu T, Sellal F, Schaeffer L, Sadoul R, Loeffler JP, René F.

Hum Mol Genet. 2016 Aug 1;25(15):3341-3360. doi: 10.1093/hmg/ddw182. Epub 2016 Jun 21.

PMID:
27329763
15.

Mutant UBQLN2 promotes toxicity by modulating intrinsic self-assembly.

Sharkey LM, Safren N, Pithadia AS, Gerson JE, Dulchavsky M, Fischer S, Patel R, Lantis G, Ashraf N, Kim JH, Meliki A, Minakawa EN, Barmada SJ, Ivanova MI, Paulson HL.

Proc Natl Acad Sci U S A. 2018 Oct 30;115(44):E10495-E10504. doi: 10.1073/pnas.1810522115. Epub 2018 Oct 17.

16.

Mutant UBQLN2P497H in motor neurons leads to ALS-like phenotypes and defective autophagy in rats.

Chen T, Huang B, Shi X, Gao L, Huang C.

Acta Neuropathol Commun. 2018 Nov 8;6(1):122. doi: 10.1186/s40478-018-0627-9.

17.

The neuropathology of FTD associated With ALS.

Mackenzie IR.

Alzheimer Dis Assoc Disord. 2007 Oct-Dec;21(4):S44-9. Review.

PMID:
18090423
18.

Ubiquilin-2 drives NF-κB activity and cytosolic TDP-43 aggregation in neuronal cells.

Picher-Martel V, Dutta K, Phaneuf D, Sobue G, Julien JP.

Mol Brain. 2015 Oct 31;8(1):71. doi: 10.1186/s13041-015-0162-6.

19.

Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia.

Deng HX, Chen W, Hong ST, Boycott KM, Gorrie GH, Siddique N, Yang Y, Fecto F, Shi Y, Zhai H, Jiang H, Hirano M, Rampersaud E, Jansen GH, Donkervoort S, Bigio EH, Brooks BR, Ajroud K, Sufit RL, Haines JL, Mugnaini E, Pericak-Vance MA, Siddique T.

Nature. 2011 Aug 21;477(7363):211-5. doi: 10.1038/nature10353.

20.

RNA-binding ability of FUS regulates neurodegeneration, cytoplasmic mislocalization and incorporation into stress granules associated with FUS carrying ALS-linked mutations.

Daigle JG, Lanson NA Jr, Smith RB, Casci I, Maltare A, Monaghan J, Nichols CD, Kryndushkin D, Shewmaker F, Pandey UB.

Hum Mol Genet. 2013 Mar 15;22(6):1193-205. doi: 10.1093/hmg/dds526. Epub 2012 Dec 20.

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