2021
Microglia regulate brain Progranulin levels through the endocytosis-lysosomal pathway
Dong T, Tejwani L, Jung Y, Kokubu H, Luttik K, Driessen TM, Lim J. Microglia regulate brain Progranulin levels through the endocytosis-lysosomal pathway. JCI Insight 2021, 6: e136147. PMID: 34618685, PMCID: PMC8663778, DOI: 10.1172/jci.insight.136147.Peer-Reviewed Original ResearchConceptsPGRN levelsNovel potential therapeutic targetFrontotemporal lobar degenerationPotential therapeutic targetNeuronal ceroid lipofuscinosisPGRN deficiencyPGRN expressionLysosomal pathwayProgranulin levelsPathological changesHaploinsufficient miceTherapeutic targetMicrogliaNeuropathological phenotypeAlzheimer's diseaseProgranulinCeroid lipofuscinosisGlycoprotein progranulinNeurodegenerative diseasesDiseaseMiceGenetic alterationsNemo-like kinaseGenetic interaction studiesGenetic variants
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