"Neuroacanthocytosis" - Overdue for a Taxonomic Update

Walker RH, Danek A.

Tremor Other Hyperkinet Mov (N Y). 2021 Jan 11;11:1. doi: 10.5334/tohm.583.

A novel ATP1A2 variant associated with severe stepwise regression, hemiplegia, epilepsy and movement disorders in two unrelated patients

Calame DG, Houck K, Lotze T, Emrick L, Parnes M.

Eur J Paediatr Neurol. 2021 Jan 16;31:21-26. doi: 10.1016/j.ejpn.2021.01.004. Online ahead of print.

Enhanced GABAergic Inhibition of Cholinergic Interneurons in the zQ175(+/-) Mouse Model of Huntington's Disease

Lim SAO, Surmeier DJ.

Front Syst Neurosci. 2021 Jan 20;14:626412. doi: 10.3389/fnsys.2020.626412. eCollection 2020.

Shape deformation analysis reveals the temporal dynamics of cell-type-specific homeostatic and pathogenic responses to mutant huntingtin

Megret L, Gris B, Sasidharan Nair S, Cevost J, Wertz M, Aaronson J, Rosinski J, Vogt TF, Wilkinson H, Heiman M, Neri C.

Elife. 2021 Feb 23;10:e64984. doi: 10.7554/eLife.64984.

Olanzapine Treatment for Chorea in a Patient With Delayed Neuropsychiatric Syndrome After Carbon Monoxide Intoxication

Wang YH, Wan FJ, Chen CY, Chang HA, Tzeng NS.

Am J Ther. 2021 Jan 15. doi: 10.1097/MJT.0000000000001336. Online ahead of print.

Super-resolution imaging reveals extrastriatal synaptic dysfunction in presymptomatic Huntington disease mice

Ravalia AS, Lau J, Barron J, Purchase S, Southwell AL, Hayden MR, Nafar F, Parsons MP.

Neurobiol Dis. 2021 Feb 5:105293. doi: 10.1016/j.nbd.2021.105293. Online ahead of print.

STIM2 Mediates Excessive Store-Operated Calcium Entry in Patient-Specific iPSC-Derived Neurons Modeling a Juvenile Form of Huntington's Disease

Vigont VA, Grekhnev DA, Lebedeva OS, Gusev KO, Volovikov EA, Skopin AY, Bogomazova AN, Shuvalova LD, Zubkova OA, Khomyakova EA, Glushankova LN, Klyushnikov SA, Illarioshkin SN, Lagarkova MA, Kaznachey

Front Cell Dev Biol. 2021 Feb 2;9:625231. doi: 10.3389/fcell.2021.625231. eCollection 2021.

Gossypol, a novel modulator of VCP, induces autophagic degradation of mutant huntingtin by promoting the formation of VCP/p97-LC3-mHTT complex

Li XJ, Zhang YY, Fu YH, Zhang H, Li HX, Li QF, Li HL, Tan RK, Jiang CX, Jiang W, Li ZX, Luo C, Lu BX, Dang YJ.

Acta Pharmacol Sin. 2021 Jan 25. doi: 10.1038/s41401-020-00605-0. Online ahead of print.

Paroxysmal Dyskinesias Revealing 3-Hydroxy-Isobutyryl-CoA Hydrolase (HIBCH) Deficiency

Spitz MA, Lenaers G, Charif M, Wirth T, Chelly J, Abi-Warde MT, Meyer P, Leboucq N, Schaefer E, Anheim M, Roubertie A.

Neuropediatrics. 2021 Jan 27. doi: 10.1055/s-0040-1722678. Online ahead of print.

Age-Related Cognitive Changes as a Function of CAG Repeat in Child and Adolescent Carriers of Mutant Huntingtin

Schultz JL, van der Plas E, Langbehn DR, Conrad AL, Nopoulos PC.

Ann Neurol. 2021 Jan 31. doi: 10.1002/ana.26039. Online ahead of print.

Optineurin deletion disrupts metabotropic glutamate receptor 5-mediated regulation of ERK1/2, GSK3β/ZBTB16, mTOR/ULK1 signaling in autophagy

Ibrahim KS, McLaren CJ, Abd-Elrahman KS, Ferguson SSG.

Biochem Pharmacol. 2021 Jan 26;185:114427. doi: 10.1016/j.bcp.2021.114427. Online ahead of print.

A fine balance between Prpf19 and Exoc7 in achieving degradation of aggregated protein and suppression of cell death in spinocerebellar ataxia type 3

Chen ZS, Huang X, Talbot K, Chan HYE.

Cell Death Dis. 2021 Feb 2;12(2):136. doi: 10.1038/s41419-021-03444-x.

Normalising life at risk of Huntington's disease. A qualitative study of backgrounds and coping strategies of fears of genetic discrimination

Wauters A, Van Hoyweghen I.

Eur J Hum Genet. 2021 Feb 16. doi: 10.1038/s41431-021-00822-z. Online ahead of print.

Exposure of R6/2 mice in an enriched environment augments P42 therapy efficacy on Huntington's disease progression

Couly S, Carles A, Denus M, Benigno-Anton L, Maschat F, Maurice T.

Neuropharmacology. 2021 Jan 28;186:108467. doi: 10.1016/j.neuropharm.2021.108467. Online ahead of print.

Enforced dimerization between XBP1s and ATF6f enhances the protective effects of the UPR in models of neurodegeneration

Vidal RL, Sepulveda D, Troncoso-Escudero P, Garcia-Huerta P, Gonzalez C, Plate L, Jerez C, Canovas J, Rivera CA, Castillo V, Cisternas M, Leal S, Martinez A, Grandjean J, Sonia D, Lashuel HA, Martin A

Mol Ther. 2021 Feb 3:S1525-0016(21)00067-8. doi: 10.1016/j.ymthe.2021.01.033. Online ahead of print.