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Items: 1 to 50 of 189

1.

ALS-Linked SOD1 Mutants Enhance Neurite Outgrowth and Branching in Adult Motor Neurons.

Osking Z, Ayers JI, Hildebrandt R, Skruber K, Brown H, Ryu D, Eukovich AR, Golde TE, Borchelt DR, Read TA, Vitriol EA.

iScience. 2019 Jan 25;11:294-304. doi: 10.1016/j.isci.2018.12.026. Epub 2018 Dec 27.

2.

Experimental Mutations in Superoxide Dismutase 1 Provide Insight into Potential Mechanisms Involved in Aberrant Aggregation in Familial Amyotrophic Lateral Sclerosis.

Crown AM, Roberts BL, Crosby K, Brown H, Ayers JI, Hart PJ, Borchelt DR.

G3 (Bethesda). 2019 Jan 8. pii: g3.200787.2018. doi: 10.1534/g3.118.200787. [Epub ahead of print]

3.

Analysis of spinal and muscle pathology in transgenic mice overexpressing wild-type and ALS-linked mutant MATR3.

Moloney C, Rayaprolu S, Howard J, Fromholt S, Brown H, Collins M, Cabrera M, Duffy C, Siemienski Z, Miller D, Borchelt DR, Lewis J.

Acta Neuropathol Commun. 2018 Dec 19;6(1):137. doi: 10.1186/s40478-018-0631-0.

4.

Aberrant accrual of BIN1 near Alzheimer's disease amyloid deposits in transgenic models.

De Rossi P, Andrew RJ, Musial TF, Buggia-Prevot V, Xu G, Ponnusamy M, Ly H, Krause SV, Rice RC, de l'Estoile V, Valin T, Salem S, Despa F, Borchelt DR, Bindokas VP, Nicholson DA, Thinakaran G.

Brain Pathol. 2018 Nov 30. doi: 10.1111/bpa.12687. [Epub ahead of print]

PMID:
30506549
5.

Loss of charge mutations in solvent exposed Lys residues of superoxide dismutase 1 do not induce inclusion formation in cultured cell models.

Crosby K, Crown AM, Roberts BL, Brown H, Ayers JI, Borchelt DR.

PLoS One. 2018 Nov 6;13(11):e0206751. doi: 10.1371/journal.pone.0206751. eCollection 2018.

6.

Changes in proteome solubility indicate widespread proteostatic disruption in mouse models of neurodegenerative disease.

Pace MC, Xu G, Fromholt S, Howard J, Crosby K, Giasson BI, Lewis J, Borchelt DR.

Acta Neuropathol. 2018 Dec;136(6):919-938. doi: 10.1007/s00401-018-1895-y. Epub 2018 Aug 23.

PMID:
30140941
7.

Targeting the Neuromuscular Junction in ALS.

Wymer J, Borchelt DR.

Neurotherapeutics. 2018 Jul;15(3):713-714. doi: 10.1007/s13311-018-0647-y. No abstract available.

PMID:
30006767
8.

Differential induction of mutant SOD1 misfolding and aggregation by tau and α-synuclein pathology.

Pace MC, Xu G, Fromholt S, Howard J, Giasson BI, Lewis J, Borchelt DR.

Mol Neurodegener. 2018 May 18;13(1):23. doi: 10.1186/s13024-018-0253-9.

9.

Targeting the accomplice to thwart the culprit: a new target for the prevention of amyloid deposition.

Borchelt DR.

J Clin Invest. 2018 May 1;128(5):1734-1736. doi: 10.1172/JCI120414. Epub 2018 Mar 30.

10.

Characterization of gene regulation and protein interaction networks for Matrin 3 encoding mutations linked to amyotrophic lateral sclerosis and myopathy.

Iradi MCG, Triplett JC, Thomas JD, Davila R, Crown AM, Brown H, Lewis J, Swanson MS, Xu G, Rodriguez-Lebron E, Borchelt DR.

Sci Rep. 2018 Mar 6;8(1):4049. doi: 10.1038/s41598-018-21371-4.

11.

Retraction Note: Transgenic mice overexpressing the ALS-linked protein Matrin 3 develop a profound muscle phenotype.

Moloney C, Rayaprolu S, Howard J, Fromholt S, Brown H, Collins M, Cabrera M, Duffy C, Siemienski Z, Miller D, Swanson MS, Notterpek L, Borchelt DR, Lewis J.

Acta Neuropathol Commun. 2017 Dec 13;5(1):97. doi: 10.1186/s40478-017-0502-0.

12.

Short Aβ peptides attenuate Aβ42 toxicity in vivo.

Moore BD, Martin J, de Mena L, Sanchez J, Cruz PE, Ceballos-Diaz C, Ladd TB, Ran Y, Levites Y, Kukar TL, Kurian JJ, McKenna R, Koo EH, Borchelt DR, Janus C, Rincon-Limas D, Fernandez-Funez P, Golde TE.

J Exp Med. 2018 Jan 2;215(1):283-301. doi: 10.1084/jem.20170600. Epub 2017 Dec 5.

13.

Prion-like Spreading in Tauopathies.

Ayers JI, Giasson BI, Borchelt DR.

Biol Psychiatry. 2018 Feb 15;83(4):337-346. doi: 10.1016/j.biopsych.2017.04.003. Epub 2017 Apr 13. Review.

14.

Quantitative Comparison of Dense-Core Amyloid Plaque Accumulation in Amyloid-β Protein Precursor Transgenic Mice.

Liu P, Reichl JH, Rao ER, McNellis BM, Huang ES, Hemmy LS, Forster CL, Kuskowski MA, Borchelt DR, Vassar R, Ashe KH, Zahs KR.

J Alzheimers Dis. 2017;56(2):743-761. doi: 10.3233/JAD-161027.

15.

Transgenic mice overexpressing the ALS-linked protein Matrin 3 develop a profound muscle phenotype.

Moloney C, Rayaprolu S, Howard J, Fromholt S, Brown H, Collins M, Cabrera M, Duffy C, Siemienski Z, Miller D, Swanson MS, Notterpek L, Borchelt DR, Lewis J.

Acta Neuropathol Commun. 2016 Nov 18;4(1):122. doi: 10.1186/s40478-016-0393-5. Retraction in: Acta Neuropathol Commun. 2017 Dec 13;5(1):97.

16.

Sex-related dimorphism in dentate gyrus atrophy and behavioral phenotypes in an inducible tTa:APPsi transgenic model of Alzheimer's disease.

Melnikova T, Park D, Becker L, Lee D, Cho E, Sayyida N, Tian J, Bandeen-Roche K, Borchelt DR, Savonenko AV.

Neurobiol Dis. 2016 Dec;96:171-185. doi: 10.1016/j.nbd.2016.08.009. Epub 2016 Aug 26.

PMID:
27569580
17.

Relationship between mutant Cu/Zn superoxide dismutase 1 maturation and inclusion formation in cell models.

Ayers JI, McMahon B, Gill S, Lelie HL, Fromholt S, Brown H, Valentine JS, Whitelegge JP, Borchelt DR.

J Neurochem. 2017 Jan;140(1):140-150. doi: 10.1111/jnc.13864. Epub 2016 Nov 25.

18.

Distinct conformers of transmissible misfolded SOD1 distinguish human SOD1-FALS from other forms of familial and sporadic ALS.

Ayers JI, Diamond J, Sari A, Fromholt S, Galaleldeen A, Ostrow LW, Glass JD, Hart PJ, Borchelt DR.

Acta Neuropathol. 2016 Dec;132(6):827-840. Epub 2016 Oct 4.

19.

Generation of a new transgenic mouse model for assessment of tau gene silencing therapies.

Fromholt S, Reitano C, Brown H, Lewis J, Borchelt DR.

Alzheimers Res Ther. 2016 Sep 5;8:36. doi: 10.1186/s13195-016-0202-1.

20.

C9orf72 BAC Mouse Model with Motor Deficits and Neurodegenerative Features of ALS/FTD.

Liu Y, Pattamatta A, Zu T, Reid T, Bardhi O, Borchelt DR, Yachnis AT, Ranum LP.

Neuron. 2016 May 4;90(3):521-34. doi: 10.1016/j.neuron.2016.04.005. Epub 2016 Apr 21.

21.

Vulnerability of newly synthesized proteins to proteostasis stress.

Xu G, Pattamatta A, Hildago R, Pace MC, Brown H, Borchelt DR.

J Cell Sci. 2016 May 1;129(9):1892-901. doi: 10.1242/jcs.176479. Epub 2016 Mar 29.

22.

Heterogeneity of Matrin 3 in the developing and aging murine central nervous system.

Rayaprolu S, D'Alton S, Crosby K, Moloney C, Howard J, Duffy C, Cabrera M, Siemienski Z, Hernandez AR, Gallego-Iradi C, Borchelt DR, Lewis J.

J Comp Neurol. 2016 Oct 1;524(14):2740-52. doi: 10.1002/cne.23986. Epub 2016 Jun 2.

23.

Prion-like propagation of mutant SOD1 misfolding and motor neuron disease spread along neuroanatomical pathways.

Ayers JI, Fromholt SE, O'Neal VM, Diamond JH, Borchelt DR.

Acta Neuropathol. 2016 Jan;131(1):103-14. doi: 10.1007/s00401-015-1514-0. Epub 2015 Dec 9.

24.

RAN Translation in Huntington Disease.

Bañez-Coronel M, Ayhan F, Tarabochia AD, Zu T, Perez BA, Tusi SK, Pletnikova O, Borchelt DR, Ross CA, Margolis RL, Yachnis AT, Troncoso JC, Ranum LP.

Neuron. 2015 Nov 18;88(4):667-77. doi: 10.1016/j.neuron.2015.10.038.

25.

Murine Aβ over-production produces diffuse and compact Alzheimer-type amyloid deposits.

Xu G, Ran Y, Fromholt SE, Fu C, Yachnis AT, Golde TE, Borchelt DR.

Acta Neuropathol Commun. 2015 Nov 14;3:72. doi: 10.1186/s40478-015-0252-9.

26.

Non-prion-type transmission in A53T α-synuclein transgenic mice: a normal component of spinal homogenates from naïve non-transgenic mice induces robust α-synuclein pathology.

Sacino AN, Ayers JI, Brooks MM, Chakrabarty P, Hudson VJ 3rd, Howard JK, Golde TE, Giasson BI, Borchelt DR.

Acta Neuropathol. 2016 Jan;131(1):151-4. doi: 10.1007/s00401-015-1505-1. Epub 2015 Nov 5. No abstract available.

27.

Subcellular Localization of Matrin 3 Containing Mutations Associated with ALS and Distal Myopathy.

Gallego-Iradi MC, Clare AM, Brown HH, Janus C, Lewis J, Borchelt DR.

PLoS One. 2015 Nov 3;10(11):e0142144. doi: 10.1371/journal.pone.0142144. eCollection 2015.

28.

Behavioral abnormalities in APPSwe/PS1dE9 mouse model of AD-like pathology: comparative analysis across multiple behavioral domains.

Janus C, Flores AY, Xu G, Borchelt DR.

Neurobiol Aging. 2015 Sep;36(9):2519-32. doi: 10.1016/j.neurobiolaging.2015.05.010. Epub 2015 May 21.

PMID:
26089165
29.

Characterization of Protein Structural Changes in Living Cells Using Time-Lapsed FTIR Imaging.

Gelfand P, Smith RJ, Stavitski E, Borchelt DR, Miller LM.

Anal Chem. 2015 Jun 16;87(12):6025-31. doi: 10.1021/acs.analchem.5b00371. Epub 2015 May 28.

30.

Substantially elevating the levels of αB-crystallin in spinal motor neurons of mutant SOD1 mice does not significantly delay paralysis or attenuate mutant protein aggregation.

Xu G, Fromholt S, Ayers JI, Brown H, Siemienski Z, Crosby KW, Mayer CA, Janus C, Borchelt DR.

J Neurochem. 2015 May;133(3):452-64. doi: 10.1111/jnc.13022. Epub 2015 Jan 26.

31.

Direct and indirect mechanisms for wild-type SOD1 to enhance the toxicity of mutant SOD1 in bigenic transgenic mice.

Xu G, Ayers JI, Roberts BL, Brown H, Fromholt S, Green C, Borchelt DR.

Hum Mol Genet. 2015 Feb 15;24(4):1019-35. doi: 10.1093/hmg/ddu517. Epub 2014 Oct 9.

32.

Gabriele Schilling (September 5, 1968-July 4, 2014).

Borchelt DR, Ross CA.

J Huntingtons Dis. 2014;3(3):225-7. doi: 10.3233/JHD-149005. No abstract available.

PMID:
25300326
33.

Experimental transmissibility of mutant SOD1 motor neuron disease.

Ayers JI, Fromholt S, Koch M, DeBosier A, McMahon B, Xu G, Borchelt DR.

Acta Neuropathol. 2014 Dec;128(6):791-803. doi: 10.1007/s00401-014-1342-7. Epub 2014 Sep 28.

PMID:
25262000
34.

Widespread and efficient transduction of spinal cord and brain following neonatal AAV injection and potential disease modifying effect in ALS mice.

Ayers JI, Fromholt S, Sinyavskaya O, Siemienski Z, Rosario AM, Li A, Crosby KW, Cruz PE, DiNunno NM, Janus C, Ceballos-Diaz C, Borchelt DR, Golde TE, Chakrabarty P, Levites Y.

Mol Ther. 2015 Jan;23(1):53-62. doi: 10.1038/mt.2014.180. Epub 2014 Sep 17.

35.

Analysis of mutant SOD1 electrophoretic mobility by Blue Native gel electrophoresis; evidence for soluble multimeric assemblies.

Brown HH, Borchelt DR.

PLoS One. 2014 Aug 14;9(8):e104583. doi: 10.1371/journal.pone.0104583. eCollection 2014.

36.

Experimental mutagenesis of huntingtin to map cleavage sites: different outcomes in cell and mouse models.

Tebbenkamp AT, Xu G, Siemienski ZB, Janus C, Fromholt SE, Brown HH, Swing D, Tessarollo L, Borchelt DR.

J Huntingtons Dis. 2014;3(1):73-86. doi: 10.3233/JHD-130087.

PMID:
25062766
37.

Intramuscular injection of α-synuclein induces CNS α-synuclein pathology and a rapid-onset motor phenotype in transgenic mice.

Sacino AN, Brooks M, Thomas MA, McKinney AB, Lee S, Regenhardt RW, McGarvey NH, Ayers JI, Notterpek L, Borchelt DR, Golde TE, Giasson BI.

Proc Natl Acad Sci U S A. 2014 Jul 22;111(29):10732-7. doi: 10.1073/pnas.1321785111. Epub 2014 Jul 7.

38.

Metal-deficient aggregates and diminished copper found in cells expressing SOD1 mutations that cause ALS.

Bourassa MW, Brown HH, Borchelt DR, Vogt S, Miller LM.

Front Aging Neurosci. 2014 Jun 16;6:110. doi: 10.3389/fnagi.2014.00110. eCollection 2014.

39.

Conformational specificity of the C4F6 SOD1 antibody; low frequency of reactivity in sporadic ALS cases.

Ayers JI, Xu G, Pletnikova O, Troncoso JC, Hart PJ, Borchelt DR.

Acta Neuropathol Commun. 2014 May 14;2:55. doi: 10.1186/2051-5960-2-55.

40.

An analysis of interactions between fluorescently-tagged mutant and wild-type SOD1 in intracellular inclusions.

Qualls DA, Crosby K, Brown H, Borchelt DR.

PLoS One. 2013 Dec 31;8(12):e83981. doi: 10.1371/journal.pone.0083981. eCollection 2013.

41.

Features of wild-type human SOD1 limit interactions with misfolded aggregates of mouse G86R Sod1.

Qualls DA, Prudencio M, Roberts BL, Crosby K, Brown H, Borchelt DR.

Mol Neurodegener. 2013 Dec 17;8:46. doi: 10.1186/1750-1326-8-46.

42.

Regenerative medicine in Alzheimer's disease.

Felsenstein KM, Candelario KM, Steindler DA, Borchelt DR.

Transl Res. 2014 Apr;163(4):432-8. doi: 10.1016/j.trsl.2013.11.001. Epub 2013 Nov 8. Review.

43.

Capsid serotype and timing of injection determines AAV transduction in the neonatal mice brain.

Chakrabarty P, Rosario A, Cruz P, Siemienski Z, Ceballos-Diaz C, Crosby K, Jansen K, Borchelt DR, Kim JY, Jankowsky JL, Golde TE, Levites Y.

PLoS One. 2013 Jun 25;8(6):e67680. doi: 10.1371/journal.pone.0067680. Print 2013.

44.

Structural similarity of wild-type and ALS-mutant superoxide dismutase-1 fibrils using limited proteolysis and atomic force microscopy.

Chan PK, Chattopadhyay M, Sharma S, Souda P, Gralla EB, Borchelt DR, Whitelegge JP, Valentine JS.

Proc Natl Acad Sci U S A. 2013 Jul 2;110(27):10934-9. doi: 10.1073/pnas.1309613110. Epub 2013 Jun 18.

45.

Unbiased screen reveals ubiquilin-1 and -2 highly associated with huntingtin inclusions.

Rutherford NJ, Lewis J, Clippinger AK, Thomas MA, Adamson J, Cruz PE, Cannon A, Xu G, Golde TE, Shaw G, Borchelt DR, Giasson BI.

Brain Res. 2013 Aug 2;1524:62-73. doi: 10.1016/j.brainres.2013.06.006. Epub 2013 Jun 15.

46.

Comment on "ApoE-directed therapeutics rapidly clear β-amyloid and reverse deficits in AD mouse models".

Price AR, Xu G, Siemienski ZB, Smithson LA, Borchelt DR, Golde TE, Felsenstein KM.

Science. 2013 May 24;340(6135):924-d. doi: 10.1126/science.1234089.

47.

Robust cytoplasmic accumulation of phosphorylated TDP-43 in transgenic models of tauopathy.

Clippinger AK, D'Alton S, Lin WL, Gendron TF, Howard J, Borchelt DR, Cannon A, Carlomagno Y, Chakrabarty P, Cook C, Golde TE, Levites Y, Ranum L, Schultheis PJ, Xu G, Petrucelli L, Sahara N, Dickson DW, Giasson B, Lewis J.

Acta Neuropathol. 2013 Jul;126(1):39-50. doi: 10.1007/s00401-013-1123-8. Epub 2013 May 11.

48.

Normal cognition in transgenic BRI2-Aβ mice.

Kim J, Chakrabarty P, Hanna A, March A, Dickson DW, Borchelt DR, Golde T, Janus C.

Mol Neurodegener. 2013 May 12;8:15. doi: 10.1186/1750-1326-8-15.

49.

Thinking laterally about neurodegenerative proteinopathies.

Golde TE, Borchelt DR, Giasson BI, Lewis J.

J Clin Invest. 2013 May;123(5):1847-55. doi: 10.1172/JCI66029. Epub 2013 May 1.

50.

Cytosolic proteins lose solubility as amyloid deposits in a transgenic mouse model of Alzheimer-type amyloidosis.

Xu G, Stevens SM Jr, Moore BD, McClung S, Borchelt DR.

Hum Mol Genet. 2013 Jul 15;22(14):2765-74. doi: 10.1093/hmg/ddt121. Epub 2013 Mar 19.

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