Artículos por temáticas

Towards a comprehensive understanding of the contributions of mitochondrial dysfunction and oxidative stress in the pathogenesis and pathophysiology of Huntington's disease.

Tobore TO.

J Neurosci Res. 2019 Nov;97(11):1455-1468. doi: 10.1002/jnr.24492. Epub 2019 Jul 15. Review.

Phenotype onset in Huntington's disease knock-in mice is correlated with the incomplete splicing of the mutant huntingtin gene.

Franich NR, Hickey MA, Zhu C, Osborne GF, Ali N, Chu T, Bove NH, Lemesre V, Lerner RP, Zeitlin SO, Howland D, Neueder A, Landles C, Bates GP, Chesselet MF.

J Neurosci Res. 2019 Dec;97(12):1590-1605. doi: 10.1002/jnr.24493. Epub 2019 Jul 7.

Conformational studies of pathogenic expanded polyglutamine protein deposits from Huntington's disease.

Matlahov I, van der Wel PC.

Exp Biol Med (Maywood). 2019 Dec;244(17):1584-1595. doi: 10.1177/1535370219856620. Epub 2019 Jun 15. No abstract available.

Visual Object Perception in Premanifest and Early Manifest Huntington's Disease.

Coppen EM, Jacobs M, van der Zwaan KF, Middelkoop HAM, Roos RAC.

Arch Clin Neuropsychol. 2019 Nov 27;34(8):1320-1328. doi: 10.1093/arclin/acz002.

Motivational Impairment is Accompanied by Corticoaccumbal Dysfunction in the BACHD-Tg5 Rat Model of Huntington's Disease.

Zlebnik NE, Gildish I, Sesia T, Fitoussi A, Cole EA, Carson BP, Cachope R, Cheer JF.

Cereb Cortex. 2019 Dec 17;29(11):4763-4774. doi: 10.1093/cercor/bhz009.

Sequelae of Lassa Fever: Postviral Cerebellar Ataxia.

Ezeomah C, Adoga A, Ihekweazu C, Paessler S, Cisneros I, Tomori O, Walker D.

Open Forum Infect Dis. 2019 Dec 3;6(12):ofz512. doi: 10.1093/ofid/ofz512. eCollection 2019 Dec. Review.

Exenatide induces frataxin expression and improves mitochondrial function in Friedreich ataxia

Igoillo-Esteve M, Oliveira AF, Cosentino C, Fantuzzi F, Demarez C, Toivonen S, Hu A, Chintawar S, Lopes M, Pachera N, Cai Y, Abdulkarim B, Rai M, Marselli L, Marchetti P, Tariq M, Jonas JC, Boscolo M,

JCI Insight. 2020 Jan 30;5(2):e134221. doi: 10.1172/jci.insight.134221.

Complexin I knockout rats exhibit a complex neurobehavioral phenotype including profound ataxia and marked deficits in lifespan

Xu Y, Zhao XM, Liu J, Wang YY, Xiong LL, He XY, Wang TH.

Pflugers Arch. 2020 Jan;472(1):117-133. doi: 10.1007/s00424-019-02337-5. Epub 2019 Dec 24.

Exploring Risk of Falls and Dynamic Unbalance in Cerebellar Ataxia by Inertial Sensor Assessment.

Caliandro P, Conte C, Iacovelli C, Tatarelli A, Castiglia SF, Reale G, Serrao M.

Sensors (Basel). 2019 Dec 17;19(24). pii: E5571. doi: 10.3390/s19245571.

Nicotinamide Pathway-Dependent Sirt1 Activation Restores Calcium Homeostasis to Achieve Neuroprotection in Spinocerebellar Ataxia Type 7

Stoyas CA, Bushart DD, Switonski PM, Ward JM, Alaghatta A, Tang MB, Niu C, Wadhwa M, Huang H, Savchenko A, Gariani K, Xie F, Delaney JR, Gaasterland T, Auwerx J, Shakkottai VG, La Spada AR.

Neuron. 2020 Feb 19;105(4):630-644.e9. doi: 10.1016/j.neuron.2019.11.019. Epub 2019 Dec 16.

Multiparametric cerebellar imaging and clinical phenotype in childhood ataxia telangiectasia

Dineen RA, Raschke F, McGlashan HL, Pszczolkowski S, Hack L, Cooper AD, Prasad M, Chow G, Whitehouse WP, Auer DP.

Neuroimage Clin. 2020;25:102110. doi: 10.1016/j.nicl.2019.102110. Epub 2019 Nov 26.

Age of onset determines intrinsic functional brain architecture in Friedreich ataxia

Naeije G, Wens V, Coquelet N, Sjøgård M, Goldman S, Pandolfo M, De Tiège XP.

Ann Clin Transl Neurol. 2020 Jan;7(1):94-104. doi: 10.1002/acn3.50966. Epub 2019 Dec 18.

Hematopoietic Stem Cell Transplantation Restores Naïve T-Cell Populations in Atm-Deficient Mice and in Preemptively Treated Patients With Ataxia-Telangiectasia.

Duecker R, Baer PC, Buecker A, Huenecke S, Pfeffermann LM, Modlich U, Bakhtiar S, Bader P, Zielen S, Schubert R.

Front Immunol. 2019 Nov 27;10:2785. doi: 10.3389/fimmu.2019.02785. eCollection 2019.

A new tool to determine the cellular metabolic landscape: nanotechnology to the study of Friedreich's ataxia.

Vannocci T, Dinarelli S, Girasole M, Pastore A, Longo G.

Sci Rep. 2019 Dec 17;9(1):19282. doi: 10.1038/s41598-019-55799-z.

Progressive Supranuclear Palsy with Predominant Cerebellar Ataxia

Ando S, Kanazawa M, Onodera O.

J Mov Disord. 2020 Jan;13(1):20-26. doi: 10.14802/jmd.19061. Epub 2019 Dec 19.