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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.


ALS-linked FUS mutations confer loss and gain of function in the nucleus by promoting excessive formation of dysfunctional paraspeckles.

An H, Skelt L, Notaro A, Highley JR, Fox AH, La Bella V, Buchman VL, Shelkovnikova TA.

Acta Neuropathol Commun. 2019 Jan 14;7(1):7. doi: 10.1186/s40478-019-0658-x.


Protective paraspeckle hyper-assembly downstream of TDP-43 loss of function in amyotrophic lateral sclerosis.

Shelkovnikova TA, Kukharsky MS, An H, Dimasi P, Alexeeva S, Shabir O, Heath PR, Buchman VL.

Mol Neurodegener. 2018 Jun 1;13(1):30. doi: 10.1186/s13024-018-0263-7.


Chronically stressed or stress-preconditioned neurons fail to maintain stress granule assembly.

Shelkovnikova TA, Dimasi P, Kukharsky MS, An H, Quintiero A, Schirmer C, Buée L, Galas MC, Buchman VL.

Cell Death Dis. 2017 May 11;8(5):e2788. doi: 10.1038/cddis.2017.199.


Modulation of p-eIF2α cellular levels and stress granule assembly/disassembly by trehalose.

Dimasi P, Quintiero A, Shelkovnikova TA, Buchman VL.

Sci Rep. 2017 Mar 9;7:44088. doi: 10.1038/srep44088.


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.


A Novel Interaction of the Catalytic Subunit of Protein Phosphatase 2A with the Adaptor Protein CIN85 Suppresses Phosphatase Activity and Facilitates Platelet Outside-in αIIbβ3 Integrin Signaling.

Khatlani T, Pradhan S, Da Q, Shaw T, Buchman VL, Cruz MA, Vijayan KV.

J Biol Chem. 2016 Aug 12;291(33):17360-8. doi: 10.1074/jbc.M115.704296. Epub 2016 Jun 22.


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.


Gamma-carboline inhibits neurodegenerative processes in a transgenic model of amyotrophic lateral sclerosis.

Bronovitsky EV, Deikin AV, Ermolkevich TG, Elyakov AB, Fedorov EN, Sadchikova ER, Goldman IL, Ovchinnikov RK, Roman AY, Khritankova IV, Kukharsky MS, Buchman VL, Bachurin SO, Ustyugov AA.

Dokl Biochem Biophys. 2015;462:189-92. doi: 10.1134/S1607672915030138. Epub 2015 Jul 12. No abstract available.


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.


Calcium-responsive transactivator (CREST) protein shares a set of structural and functional traits with other proteins associated with amyotrophic lateral sclerosis.

Kukharsky MS, Quintiero A, Matsumoto T, Matsukawa K, An H, Hashimoto T, Iwatsubo T, Buchman VL, Shelkovnikova TA.

Mol Neurodegener. 2015 Apr 10;10:20. doi: 10.1186/s13024-015-0014-y.


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.


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.


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.


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.


Recruitment into stress granules prevents irreversible aggregation of FUS protein mislocalized to the cytoplasm.

Shelkovnikova TA, Robinson HK, Connor-Robson N, Buchman VL.

Cell Cycle. 2013 Oct 1;12(19):3194-202. doi: 10.4161/cc.26241. Epub 2013 Sep 4.


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.


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.


Identification of Nedd4 E3 ubiquitin ligase as a binding partner and regulator of MAK-V protein kinase.

Kalinichenko SV, Itoh K, Korobko EV, Sokol SY, Buchman VL, Korobko IV.

PLoS One. 2012;7(6):e39505. doi: 10.1371/journal.pone.0039505. Epub 2012 Jun 20.


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.


Deletion of alpha-synuclein decreases impulsivity in mice.

Peña-Oliver Y, Buchman VL, Dalley JW, Robbins TW, Schumann G, Ripley TL, King SL, Stephens DN.

Genes Brain Behav. 2012 Mar;11(2):137-46. doi: 10.1111/j.1601-183X.2011.00758.x. Epub 2011 Dec 29.


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.


Myelination transition zone astrocytes are constitutively phagocytic and have synuclein dependent reactivity in glaucoma.

Nguyen JV, Soto I, Kim KY, Bushong EA, Oglesby E, Valiente-Soriano FJ, Yang Z, Davis CH, Bedont JL, Son JL, Wei JO, Buchman VL, Zack DJ, Vidal-Sanz M, Ellisman MH, Marsh-Armstrong N.

Proc Natl Acad Sci U S A. 2011 Jan 18;108(3):1176-81. doi: 10.1073/pnas.1013965108. Epub 2011 Jan 3.


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.


αβγ-Synuclein triple knockout mice reveal age-dependent neuronal dysfunction.

Greten-Harrison B, Polydoro M, Morimoto-Tomita M, Diao L, Williams AM, Nie EH, Makani S, Tian N, Castillo PE, Buchman VL, Chandra SS.

Proc Natl Acad Sci U S A. 2010 Nov 9;107(45):19573-8. doi: 10.1073/pnas.1005005107. Epub 2010 Oct 25.


α-Synuclein and dopamine at the crossroads of Parkinson's disease.

Venda LL, Cragg SJ, Buchman VL, Wade-Martins R.

Trends Neurosci. 2010 Dec;33(12):559-68. doi: 10.1016/j.tins.2010.09.004. Epub 2010 Oct 18. Review.


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.


Emerging roles of Ruk/CIN85 in vesicle-mediated transport, adhesion, migration and malignancy.

Havrylov S, Redowicz MJ, Buchman VL.

Traffic. 2010 Jun;11(6):721-31. doi: 10.1111/j.1600-0854.2010.01061.x. Epub 2010 Mar 17. Review.


Lack of involvement of alpha-synuclein in unconditioned anxiety in mice.

Peña-Oliver Y, Buchman VL, Stephens DN.

Behav Brain Res. 2010 Jun 19;209(2):234-40. doi: 10.1016/j.bbr.2010.01.049. Epub 2010 Feb 4.


Differential expression of sarcoplasmic and myofibrillar proteins of rat soleus muscle during denervation atrophy.

Sato Y, Shimizu M, Mizunoya W, Wariishi H, Tatsumi R, Buchman VL, Ikeuchi Y.

Biosci Biotechnol Biochem. 2009 Aug;73(8):1748-56. Epub 2009 Aug 7.


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.


Generation of mutant mice with targeted disruption of two members of the d4 gene family: neuro-d4 and cer-d4.

Mertsalov IB, Ninkina NN, Wanless JS, Buchman VL, Korochkin LI, Kulikova DA.

Dokl Biochem Biophys. 2008 Mar-Apr;419:65-8. No abstract available.


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