2020
Nemo-like kinase reduces mutant huntingtin levels and mitigates Huntington’s disease
Jiang M, Zhang X, Liu H, LeBron J, Alexandris A, Peng Q, Gu H, Yang F, Li Y, Wang R, Hou Z, Arbez N, Ren Q, Dong JL, Whela E, Wang R, Ratovitski T, Troncoso JC, Mori S, Ross CA, Lim J, Duan W. Nemo-like kinase reduces mutant huntingtin levels and mitigates Huntington’s disease. Human Molecular Genetics 2020, 29: 1340-1352. PMID: 32242231, PMCID: PMC7254850, DOI: 10.1093/hmg/ddaa061.Peer-Reviewed Original ResearchConceptsBrain atrophyHD miceNemo-like kinaseMHTT levelsHD mouse modelsNew molecular targetsHD human brainHuntingtin proteinEffect of NLKMouse striatal cellsFurther mechanistic studiesActivity-dependent mannerHTT protein levelsMouse modelAdult brainStriatal cellsProtective roleMutant Htt aggregationAmino acids 120Huntington's diseaseMutant huntingtin levelsMolecular targetsHuntingtin levelsProtein levelsBrain
2015
Nemo-like kinase is a novel regulator of spinal and bulbar muscular atrophy
Todd TW, Kokubu H, Miranda HC, Cortes CJ, La Spada AR, Lim J. Nemo-like kinase is a novel regulator of spinal and bulbar muscular atrophy. ELife 2015, 4: e08493. PMID: 26308581, PMCID: PMC4577982, DOI: 10.7554/elife.08493.Peer-Reviewed Original ResearchConceptsNemo-like kinaseMuscular atrophyExact pathogenic mechanismProgressive neuromuscular diseaseAndrogen receptor proteinSBMA phenotypePathogenic mechanismsDisease pathogenesisNeuromuscular diseaseGene transcriptionTherapy developmentAtrophySBMAAR fragmentReceptor proteinPolyglutamine expansionMolecular mechanismsNovel regulatorNovel avenuesToxicityPathogenesisDiseaseMice
2013
Polyglutamine Disease Toxicity Is Regulated by Nemo-like Kinase in Spinocerebellar Ataxia Type 1
Ju H, Kokubu H, Todd TW, Kahle JJ, Kim S, Richman R, Chirala K, Orr HT, Zoghbi HY, Lim J. Polyglutamine Disease Toxicity Is Regulated by Nemo-like Kinase in Spinocerebellar Ataxia Type 1. Journal Of Neuroscience 2013, 33: 9328-9336. PMID: 23719801, PMCID: PMC3710458, DOI: 10.1523/jneurosci.3465-12.2013.Peer-Reviewed Original ResearchMeSH KeywordsAnimalsAnimals, Genetically ModifiedAtaxin-1AtaxinsBehavior, AnimalBlotting, WesternBrainCerebellumChromatography, GelDrosophila melanogasterFemaleGene ExpressionHEK293 CellsHeredodegenerative Disorders, Nervous SystemHumansImmunoprecipitationMiceMice, Inbred C57BLMice, TransgenicMitogen-Activated Protein KinasesNerve Tissue ProteinsNuclear ProteinsPeptidesPhosphorylationProtein Serine-Threonine KinasesSpinocerebellar Ataxias