Microbiome profiling reveals gut dysbiosis in a transgenic mouse model of Huntington's disease.

Kong G, Cao KL, Judd LM, Li S, Renoir T, Hannan AJ.

Neurobiol Dis. 2020 Feb;135:104268. doi: 10.1016/j.nbd.2018.09.001. Epub 2018 Sep 5.

mHTT Seeding Activity: A Marker of Disease Progression and Neurotoxicity in Models of Huntington's Disease.

Ast A, Buntru A, Schindler F, Hasenkopf R, Schulz A, Brusendorf L, Klockmeier K, Grelle G, McMahon B, Niederlechner H, Jansen I, Diez L, Edel J, Boeddrich A, Franklin SA, Baldo B, Schnoegl S, Kunz S,

Mol Cell. 2018 Sep 6;71(5):675-688.e6. doi: 10.1016/j.molcel.2018.07.032.

Biological and clinical manifestations of juvenile Huntington's disease: a retrospective analysis.

Fusilli C, Migliore S, Mazza T, Consoli F, De Luca A, Barbagallo G, Ciammola A, Gatto EM, Cesarini M, Etcheverry JL, Parisi V, Al-Oraimi M, Al-Harrasi S, Al-Salmi Q, Marano M, Vonsattel JG, Sabatini U

Lancet Neurol. 2018 Sep 19. pii: S1474-4422(18)30294-1. doi: 10.1016/S1474-4422(18)30294-1. [Epub ahead of print]

Huntington's disease leads to decrease of GABA-A tonic subunits in the D2 neostriatal pathway and their relocalization into the synaptic cleft.

Rosas-Arellano A, Tejeda-Guzmán C, Lorca-Ponce E, Palma-Tirado L, Mantellero CA, Rojas P, Missirlis F, Castro MA.

Neurobiol Dis. 2018 Feb;110:142-153. doi: 10.1016/j.nbd.2017.11.010. Epub 2017 Dec 2.

High and Low Levels of an NTRK2-Driven Genetic Profile Affect Motor- and Cognition-Associated Frontal Gray Matter in Prodromal Huntington's Disease.

Ciarochi JA, Liu J, Calhoun V, Johnson H, Misiura M, Bockholt HJ, Espinoza FA, Caprihan A, Plis S, Turner JA, Paulsen JS; PREDICT-HD Investigators and Coordinators of the Huntington Study Group..

Brain Sci. 2018 Jun 22;8(7). pii: E116. doi: 10.3390/brainsci8070116.

SIRT1 is increased in affected brain regions and hypothalamic metabolic pathways are altered in Huntington disease.

Baldo B, Gabery S, Soylu-Kucharz R, Cheong RY, Henningsen JB, Englund E, McLean C, Kirik D, Halliday G, Petersén Å.

Neuropathol Appl Neurobiol. 2018 Jul 18. doi: 10.1111/nan.12514. [Epub ahead of print]

Mutant huntingtin induces iron overload via up-regulating IRP1 in Huntington's disease.

Niu L, Ye C, Sun Y, Peng T, Yang S, Wang W, Li H.

Cell Biosci. 2018 Jul 4;8:41. doi: 10.1186/s13578-018-0239-x. eCollection 2018.

A transgenic minipig model of Huntington's disease shows early signs of behavioral and molecular pathologies.

Askeland G, Rodinova M, Štufková H, Dosoudilova Z, Baxa M, Smatlikova P, Bohuslavova B, Klempir J, Nguyen TD, Kuśnierczyk A, Bjørås M, Klungland A, Hansikova H, Ellederova Z, Eide L.

Dis Model Mech. 2018 Sep 26. pii: dmm.035949. doi: 10.1242/dmm.035949. [Epub ahead of print]

Corticostriatal network dysfunction in huntington's disease: Deficits in neural processing, glutamate transport, and ascorbate release.

Rebec GV.

CNS Neurosci Ther. 2018 Feb 21. doi: 10.1111/cns.12828. [Epub ahead of print] Review.

Rare case of chorea-hyperglycaemia-basal ganglia (C-H-BG) syndrome.

Ahmad S, Mohan Babu P, Shenbagaraj L, George L.

BMJ Case Rep. 2018 Apr 17;2018. pii: bcr-2017-223920. doi: 10.1136/bcr-2017-223920.

Characterization of adenine nucleotide metabolism in the cellular model of Huntington's disease.

Toczek M, Pierzynowska K, Kutryb-Zajac B, Gaffke L, Slominska EM, Wegrzyn G, Smolenski RT.

Nucleosides Nucleotides Nucleic Acids. 2018 Dec 27:1-9. doi: 10.1080/15257770.2018.1481508. [Epub ahead of print]

Chorea-Acanthocytosis Presenting as Autosomal Recessive Epilepsy in a Family With a Novel VPS13A Mutation.

Weber J, Frings L, Rijntjes M, Urbach H, Fischer J, Weiller C, Meyer PT, Klebe S.

Front Neurol. 2019 Jan 9;9:1168. doi: 10.3389/fneur.2018.01168. eCollection 2018.

Increased oxidative stress and CaMKII activity contribute to electro-mechanical defects in cardiomyocytes from a murine model of Huntington's disease.

Joviano-Santos JV, Santos-Miranda A, Botelho AFM, de Jesus ICG, Andrade JN, de Oliveira Barreto T, Magalhães-Gomes MPS, Valadão PAC, Dos Santos Cruz J, Melo MM, Guatimosim S, Guatimosim C.

FEBS J. 2018 Nov 19. doi: 10.1111/febs.14706. [Epub ahead of print]

Evaluating the current state of the art of Huntington disease research: a scientometric analysis.

Barboza LA, Ghisi NC.

Braz J Med Biol Res. 2018 Jan 11;51(3):e6299. doi: 10.1590/1414-431X20176299.

Cognitive Performance After a One-Year Multidisciplinary Intensive Rehabilitation Program for Huntington's Disease: An Observational Study.

van Walsem MR, Piira A, Mikalsen G, Fossmo HL, Howe EI, Knutsen SF, Frich JC.

J Huntingtons Dis. 2018 Sep 29. doi: 10.3233/JHD-180294. [Epub ahead of print]