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Gamma-Carbolines Derivatives As Promising Agents for the Development of Pathogenic Therapy for Proteinopathy.

Skvortsova VI, Bachurin SO, Ustyugov AA, Kukharsky MS, Deikin AV, Buchman VL, Ninkina NN.

Acta Naturae. 2018 Oct-Dec;10(4):59-62.


Zinc Induces Temperature-Dependent Reversible Self-Assembly of Tau.

Roman AY, Devred F, Byrne D, La Rocca R, Ninkina NN, Peyrot V, Tsvetkov PO.

J Mol Biol. 2019 Feb 15;431(4):687-695. doi: 10.1016/j.jmb.2018.12.008. Epub 2018 Dec 21.


Zinc binding to RNA recognition motif of TDP-43 induces the formation of amyloid-like aggregates.

Garnier C, Devred F, Byrne D, Puppo R, Roman AY, Malesinski S, Golovin AV, Lebrun R, Ninkina NN, Tsvetkov PO.

Sci Rep. 2017 Jul 28;7(1):6812. doi: 10.1038/s41598-017-07215-7.


Detection of autoantibodies to potentially amyloidogenic protein, gamma-synuclein, in the serum of patients with amyotrophic lateral sclerosis and cerebral circulatory disorders.

Roman AY, Kovrazhkina EA, Razinskaya OD, Kukharsky MS, Maltsev AV, Ovchinnikov RK, Lytkina OA, Smirnov AP, Moskovtsev AA, Borodina YV, Surguchov AP, Ustyugov AA, Ninkina NN, Skvortsova VI.

Dokl Biochem Biophys. 2017 Jan;472(1):64-67. doi: 10.1134/S1607672917010197. Epub 2017 Apr 19.


Generation of mouse lines with conditionally or constitutively inactivated Snca gene and Rosa26-stop-lacZ reporter located in cis on the mouse chromosome 6.

Roman AY, Limorenko G, Ustyugov AA, Tarasova TV, Lysikova EA, Buchman VL, Ninkina N.

Transgenic Res. 2017 Apr;26(2):301-307. doi: 10.1007/s11248-016-9995-8. Epub 2016 Nov 12.


Monomeric Alpha-Synuclein Exerts a Physiological Role on Brain ATP Synthase.

Ludtmann MH, Angelova PR, Ninkina NN, Gandhi S, Buchman VL, Abramov AY.

J Neurosci. 2016 Oct 12;36(41):10510-10521.


Combinational losses of synucleins reveal their differential requirements for compensating age-dependent alterations in motor behavior and dopamine metabolism.

Connor-Robson N, Peters OM, Millership S, Ninkina N, Buchman VL.

Neurobiol Aging. 2016 Oct;46:107-12. doi: 10.1016/j.neurobiolaging.2016.06.020. Epub 2016 Jul 4.


Induction of de novo α-synuclein fibrillization in a neuronal model for Parkinson's disease.

Fares MB, Maco B, Oueslati A, Rockenstein E, Ninkina N, Buchman VL, Masliah E, Lashuel HA.

Proc Natl Acad Sci U S A. 2016 Feb 16;113(7):E912-21. doi: 10.1073/pnas.1512876113. Epub 2016 Feb 2.


A novel resource for studying function and dysfunction of α-synuclein: mouse lines for modulation of endogenous Snca gene expression.

Ninkina N, Connor-Robson N, Ustyugov AA, Tarasova TV, Shelkovnikova TA, Buchman VL.

Sci Rep. 2015 Nov 13;5:16615. doi: 10.1038/srep16615.


C9ORF72 hexanucleotide repeat expansion in ALS patients from the Central European Russia population.

Abramycheva NY, Lysogorskaia EV, Stepanova MS, Zakharova MN, Kovrazhkina EA, Razinskaya OD, Smirnov AP, Maltsev AV, Ustyugov AA, Kukharsky MS, Khritankova IV, Bachurin SO, Cooper-Knock J, Buchman VL, Illarioshkin SN, Skvortsova VI, Ninkina N.

Neurobiol Aging. 2015 Oct;36(10):2908.e5-9. doi: 10.1016/j.neurobiolaging.2015.07.004. Epub 2015 Jul 9.


Control of ventricular excitability by neurons of the dorsal motor nucleus of the vagus nerve.

Machhada A, Ang R, Ackland GL, Ninkina N, Buchman VL, Lythgoe MF, Trapp S, Tinker A, Marina N, Gourine AV.

Heart Rhythm. 2015 Nov;12(11):2285-93. doi: 10.1016/j.hrthm.2015.06.005. Epub 2015 Jun 5.


Early lethality and neuronal proteinopathy in mice expressing cytoplasm-targeted FUS that lacks the RNA recognition motif.

Robinson HK, Deykin AV, Bronovitsky EV, Ovchinnikov RK, Ustyugov AA, Shelkovnikova TA, Kukharsky MS, Ermolkevich TG, Goldman IL, Sadchikova ER, Kovrazhkina EA, Bachurin SO, Buchman VL, Ninkina NN.

Amyotroph Lateral Scler Frontotemporal Degener. 2015;16(5-6):402-9. doi: 10.3109/21678421.2015.1040994. Epub 2015 May 20.


Hunk/Mak-v is a negative regulator of intestinal cell proliferation.

Reed KR, Korobko IV, Ninkina N, Korobko EV, Hopkins BR, Platt JL, Buchman V, Clarke AR.

BMC Cancer. 2015 Mar 8;15:110. doi: 10.1186/s12885-015-1087-2.


Gamma-synuclein pathology in amyotrophic lateral sclerosis.

Peters OM, Shelkovnikova T, Highley JR, Cooper-Knock J, Hortobágyi T, Troakes C, Ninkina N, Buchman VL.

Ann Clin Transl Neurol. 2015 Jan;2(1):29-37. doi: 10.1002/acn3.143. Epub 2014 Dec 12.


Role of synucleins in traumatic brain injury — an experimental in vitro and in vivo study in mice.

Surgucheva I, He S, Rich MC, Sharma R, Ninkina NN, Stahel PF, Surguchov A.

Mol Cell Neurosci. 2014 Nov;63:114-23.


[A mice model of amyotrophic lateral sclerosis expressing mutant human FUS protein].

Deĭkin AV, Kovrazhkina EA, Ovchinnikov RK, Bronovitskiĭ EV, Razinskaia OD, Smirnov AP, Ermolkevich TG, Eliakov AB, Popov AN, Fedorov EN, Lytkina OA, Kukharskiĭ MS, Tarasova TV, Shelkovnikova TA, Ustiugov AA, Ninkina NN, Gol'dman IL, Sadchikova ER, Bachurin SO, Skvortsova VI.

Zh Nevrol Psikhiatr Im S S Korsakova. 2014;114(8):62-9. Russian.


[Study into molecular targets of a neuroprotective compound dimebon using a transgenic mice line].

Shelkovnikova TA, Ustiugov AA, Kokhan VS, Tarasova TV, Medvedeva VK, Khritankova IV, Bachurin SO, Ninkina NN.

Biomed Khim. 2014 May-Jun;60(3):354-63. Russian.


Multistep process of FUS aggregation in the cell cytoplasm involves RNA-dependent and RNA-independent mechanisms.

Shelkovnikova TA, Robinson HK, Southcombe JA, Ninkina N, Buchman VL.

Hum Mol Genet. 2014 Oct 1;23(19):5211-26. doi: 10.1093/hmg/ddu243. Epub 2014 May 19.


[Modeling of lateral amyotrophic sclerosis: a transgenic method].

Bachurin SO, Ninkina NN, Tarasova TV, Shelkovnikova TV, Kovrazhkina EA, Smirnov AP, Razinskaia OD, Skvortsova VI.

Zh Nevrol Psikhiatr Im S S Korsakova. 2013;113(10):74-9. Review. Russian. No abstract available.


Compromised paraspeckle formation as a pathogenic factor in FUSopathies.

Shelkovnikova TA, Robinson HK, Troakes C, Ninkina N, Buchman VL.

Hum Mol Genet. 2014 May 1;23(9):2298-312. doi: 10.1093/hmg/ddt622. Epub 2013 Dec 11.


[Modeling of lateral amyotrophic sclerosis: a non-genetic method].

Bachurin SO, Ninkina NN, Tarasova TV, Shelkovnikova TV, Kovrazhkina EA, Smirnov AP, Razinskaya O, Skvortsova VI.

Zh Nevrol Psikhiatr Im S S Korsakova. 2013;113(9):86-9. Review. Russian. No abstract available.


C9ORF72 transcription in a frontotemporal dementia case with two expanded alleles.

Cooper-Knock J, Higginbottom A, Connor-Robson N, Bayatti N, Bury JJ, Kirby J, Ninkina N, Buchman VL, Shaw PJ.

Neurology. 2013 Nov 5;81(19):1719-21. doi: 10.1212/01.wnl.0000435295.41974.2e. Epub 2013 Oct 9.


γ-synuclein is a novel player in the control of body lipid metabolism.

Millership S, Ninkina N, Rochford JJ, Buchman VL.

Adipocyte. 2013 Oct 1;2(4):276-80. doi: 10.4161/adip.25162. Epub 2013 May 28.


Therapeutic effect of exogenous hsp70 in mouse models of Alzheimer's disease.

Bobkova NV, Garbuz DG, Nesterova I, Medvinskaya N, Samokhin A, Alexandrova I, Yashin V, Karpov V, Kukharsky MS, Ninkina NN, Smirnov AA, Nudler E, Evgen'ev M.

J Alzheimers Dis. 2014;38(2):425-35. doi: 10.3233/JAD-130779.


Endogenous alpha-synuclein influences the number of dopaminergic neurons in mouse substantia nigra.

Garcia-Reitboeck P, Anichtchik O, Dalley JW, Ninkina N, Tofaris GK, Buchman VL, Spillantini MG.

Exp Neurol. 2013 Oct;248:541-5. doi: 10.1016/j.expneurol.2013.07.015. Epub 2013 Aug 8.


Fused in sarcoma (FUS) protein lacking nuclear localization signal (NLS) and major RNA binding motifs triggers proteinopathy and severe motor phenotype in transgenic mice.

Shelkovnikova TA, Peters OM, Deykin AV, Connor-Robson N, Robinson H, Ustyugov AA, Bachurin SO, Ermolkevich TG, Goldman IL, Sadchikova ER, Kovrazhkina EA, Skvortsova VI, Ling SC, Da Cruz S, Parone PA, Buchman VL, Ninkina NN.

J Biol Chem. 2013 Aug 30;288(35):25266-74. doi: 10.1074/jbc.M113.492017. Epub 2013 Jul 18.


Chronic administration of Dimebon does not ameliorate amyloid-β pathology in 5xFAD transgenic mice.

Peters OM, Shelkovnikova T, Tarasova T, Springe S, Kukharsky MS, Smith GA, Brooks S, Kozin SA, Kotelevtsev Y, Bachurin SO, Ninkina N, Buchman VL.

J Alzheimers Dis. 2013;36(3):589-96. doi: 10.3233/JAD-130071.


Simultaneous and independent detection of C9ORF72 alleles with low and high number of GGGGCC repeats using an optimised protocol of Southern blot hybridisation.

Buchman VL, Cooper-Knock J, Connor-Robson N, Higginbottom A, Kirby J, Razinskaya OD, Ninkina N, Shaw PJ.

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


Increased lipolysis and altered lipid homeostasis protect γ-synuclein-null mutant mice from diet-induced obesity.

Millership S, Ninkina N, Guschina IA, Norton J, Brambilla R, Oort PJ, Adams SH, Dennis RJ, Voshol PJ, Rochford JJ, Buchman VL.

Proc Natl Acad Sci U S A. 2012 Dec 18;109(51):20943-8. doi: 10.1073/pnas.1210022110. Epub 2012 Dec 3. Erratum in: Proc Natl Acad Sci U S A. 2013 Mar 26;110(13):5269. Brambilla, Ricardo [corrected to Brambilla, Riccardo].


Contrasting effects of α-synuclein and γ-synuclein on the phenotype of cysteine string protein α (CSPα) null mutant mice suggest distinct function of these proteins in neuronal synapses.

Ninkina N, Peters OM, Connor-Robson N, Lytkina O, Sharfeddin E, Buchman VL.

J Biol Chem. 2012 Dec 28;287(53):44471-7. doi: 10.1074/jbc.M112.422402. Epub 2012 Nov 5.


Chronic administration of dimebon ameliorates pathology in TauP301S transgenic mice.

Peters OM, Connor-Robson N, Sokolov VB, Aksinenko AY, Kukharsky MS, Bachurin SO, Ninkina N, Buchman VL.

J Alzheimers Dis. 2013;33(4):1041-9. doi: 10.3233/JAD-2012-121732.


[Proteinopathies--forms of neurodegenerative disorders with protein aggregation-based pathology].

Shelkovnikova TA, Kulikova AA, Tsvetkov FO, Peters O, Bachurin SO, Bukhman VL, Ninkina NN.

Mol Biol (Mosk). 2012 May-Jun;46(3):402-15. Review. Russian.


Dimebon reduces the levels of aggregated amyloidogenic protein forms in detergent-insoluble fractions in vivo.

Ustyugov AA, Shelkovnikova TA, Kokhan VS, Khritankova IV, Peters O, Buchman VL, Bachurin SO, Ninkina NN.

Bull Exp Biol Med. 2012 Apr;152(6):731-3. English, Russian.


Selective pattern of motor system damage in gamma-synuclein transgenic mice mirrors the respective pathology in amyotrophic lateral sclerosis.

Peters OM, Millership S, Shelkovnikova TA, Soto I, Keeling L, Hann A, Marsh-Armstrong N, Buchman VL, Ninkina N.

Neurobiol Dis. 2012 Oct;48(1):124-31. doi: 10.1016/j.nbd.2012.06.016. Epub 2012 Jun 30.


Impaired spatial and working memory in ageing mice with targeted inactivation of α-synuclein gene.

Kokhan VS, Van'kin GI, Ninkina NN, Shelkovnikova TA, Bachurin SO.

Dokl Biol Sci. 2011 Nov-Dec;441:354-6. doi: 10.1134/S0012496611060081. Epub 2012 Jan 7. No abstract available.


Dimebon slows progression of proteinopathy in γ-synuclein transgenic mice.

Bachurin SO, Shelkovnikova TA, Ustyugov AA, Peters O, Khritankova I, Afanasieva MA, Tarasova TV, Alentov II, Buchman VL, Ninkina NN.

Neurotox Res. 2012 Jul;22(1):33-42. doi: 10.1007/s12640-011-9299-y. Epub 2011 Dec 17.


FUS gene mutations associated with familiar forms of amyotrophic lateral sclerosis affect cellular localization and aggregation properties of the encoded protein.

Shelkovnikova TA, Ustyugov AA, Smirnov AP, Skvortsova VI, Buchman VL, Bachurin SO, Ninkina NN.

Dokl Biochem Biophys. 2011 May-Jun;438:123-6. doi: 10.1134/S1607672911030045. Epub 2011 Jul 3. No abstract available.


Functional alterations to the nigrostriatal system in mice lacking all three members of the synuclein family.

Anwar S, Peters O, Millership S, Ninkina N, Doig N, Connor-Robson N, Threlfell S, Kooner G, Deacon RM, Bannerman DM, Bolam JP, Chandra SS, Cragg SJ, Wade-Martins R, Buchman VL.

J Neurosci. 2011 May 18;31(20):7264-74. doi: 10.1523/JNEUROSCI.6194-10.2011.


Dimebon does not ameliorate pathological changes caused by expression of truncated (1-120) human alpha-synuclein in dopaminergic neurons of transgenic mice.

Shelkovnikova TA, Ustyugov AA, Millership S, Peters O, Anichtchik O, Spillantini MG, Buchman VL, Bachurin SO, Ninkina NN.

Neurodegener Dis. 2011;8(6):430-7. doi: 10.1159/000324989. Epub 2011 May 12.


[New aspects of the pathogenesis of lateral amyotrophic sclerosis].

Skvortsova VI, Bachurin SO, Razinskaia OD, Smirnov AP, Kovrazhkina EA, Pochigaeva KI, Ninkina NN, Shelkovnikova TA, Ustiugov AA.

Zh Nevrol Psikhiatr Im S S Korsakova. 2011;111(2):4-9. Russian. No abstract available.


[Targeted inactivation of gamma-synuclein gene affects anxiety and exploratory behaviour of mice].

Kokhan VS, Bolkunov AV, Ustiugov AA, Van'kin GI, Shelkovnikova TA, Redkozubova OM, Strekalova TV, Bukhman VL, Ninkina NN, Bachurin SO.

Zh Vyssh Nerv Deiat Im I P Pavlova. 2011 Jan-Feb;61(1):85-93. Russian.


Pro-survival activity of the MAK-V protein kinase in PC12 cells.

Korobko IV, Kalinichenko SV, Korobko EV, Ninkina NN, Kiselev SL, Buchman VL.

Cell Cycle. 2010 Oct 15;9(20):4248-9. No abstract available.


Lipid classes and fatty acid patterns are altered in the brain of γ-synuclein null mutant mice.

Guschina I, Millership S, O'Donnell V, Ninkina N, Harwood J, Buchman V.

Lipids. 2011 Feb;46(2):121-30. doi: 10.1007/s11745-010-3486-0. Epub 2010 Oct 21.


Hindering of proteinopathy-induced neurodegeneration as a new mechanism of action for neuroprotectors and cognition enhancing compounds.

Bachurin SO, Ustyugov AA, Peters O, Shelkovnikova TA, Buchman VL, Ninkina NN.

Dokl Biochem Biophys. 2009 Sep-Oct;428:235-8. No abstract available.


Methylene blue and dimebon inhibit aggregation of TDP-43 in cellular models.

Yamashita M, Nonaka T, Arai T, Kametani F, Buchman VL, Ninkina N, Bachurin SO, Akiyama H, Goedert M, Hasegawa M.

FEBS Lett. 2009 Jul 21;583(14):2419-24. doi: 10.1016/j.febslet.2009.06.042. Epub 2009 Jun 26.


Gamma-synucleinopathy: neurodegeneration associated with overexpression of the mouse protein.

Ninkina N, Peters O, Millership S, Salem H, van der Putten H, Buchman VL.

Hum Mol Genet. 2009 May 15;18(10):1779-94. doi: 10.1093/hmg/ddp090. Epub 2009 Feb 26.


Absence of alpha-synuclein affects dopamine metabolism and synaptic markers in the striatum of aging mice.

Al-Wandi A, Ninkina N, Millership S, Williamson SJ, Jones PA, Buchman VL.

Neurobiol Aging. 2010 May;31(5):796-804. doi: 10.1016/j.neurobiolaging.2008.11.001. Epub 2008 Dec 20.


Modulation of alpha-synuclein expression in transgenic animals for modelling synucleinopathies--is the juice worth the squeeze?

Buchman VL, Ninkina N.

Neurotox Res. 2008 Dec;14(4):329-41. doi: 10.1007/BF03033857. Review.


[Modelling synucleinopathies in genetically modified animals--successes and failures].

Ninkina NN, Ustiugov AA, Bukhman VL.

Mol Biol (Mosk). 2008 Sep-Oct;42(5):840-55. Review. Russian.


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