2020
Chaperonin-assisted protein folding: a chronologue
Horwich AL, Fenton WA. Chaperonin-assisted protein folding: a chronologue. Quarterly Reviews Of Biophysics 2020, 53: e4. PMID: 32070442, DOI: 10.1017/s0033583519000143.Peer-Reviewed Original ResearchMeSH KeywordsAdenosine TriphosphateAmino AcidsAnimalsCarbon DioxideChaperoninsCytosolDimerizationHeat-Shock ProteinsHumansHydrophobic and Hydrophilic InteractionsKineticsMiceMitochondriaMutationNeurosporaProtein ConformationProtein DenaturationProtein FoldingRibonuclease, PancreaticRibulose-Bisphosphate CarboxylaseSurface PropertiesTemperature
2017
Transfer of pathogenic and nonpathogenic cytosolic proteins between spinal cord motor neurons in vivo in chimeric mice
Thomas EV, Fenton WA, McGrath J, Horwich AL. Transfer of pathogenic and nonpathogenic cytosolic proteins between spinal cord motor neurons in vivo in chimeric mice. Proceedings Of The National Academy Of Sciences Of The United States Of America 2017, 114: e3139-e3148. PMID: 28348221, PMCID: PMC5393223, DOI: 10.1073/pnas.1701465114.Peer-Reviewed Original ResearchConceptsMotor neuronsChimeric miceSpinal cord motor neuronsCranial nerve motor nucleiDouble fluorescenceMammalian nervous systemMo of ageGray matter oligodendrocytesCytosolic proteinsExtraocular nucleiMotor nucleusSpinal cordNervous systemNeuronsMiceSuperoxide dismutase 1 proteinEight-cell embryosPathogenic proteinsOligodendrocytesDismutase 1 proteinThird chimeraChimeric progenyRecent studiesEGFP chimerasCells
2016
Reduced high-frequency motor neuron firing, EMG fractionation, and gait variability in awake walking ALS mice
Hadzipasic M, Ni W, Nagy M, Steenrod N, McGinley MJ, Kaushal A, Thomas E, McCormick DA, Horwich AL. Reduced high-frequency motor neuron firing, EMG fractionation, and gait variability in awake walking ALS mice. Proceedings Of The National Academy Of Sciences Of The United States Of America 2016, 113: e7600-e7609. PMID: 27821773, PMCID: PMC5127366, DOI: 10.1073/pnas.1616832113.Peer-Reviewed Original ResearchConceptsALS miceAmyotrophic lateral sclerosisAcute spinal cord slicesSingle-unit extracellular recordingsWhole-cell patch-clamp recordingsLoss of neuronsMotor neuron lossMotor neuron firingSpinal cord slicesPatch-clamp recordingsHigh-frequency firingStep variabilityLethal neurodegenerative diseaseNeuron lossCord slicesSpinal cordLeg flexorsLateral sclerosisGait variabilityVivo effectsClamp recordingsExtracellular recordingsEMG patternsMutant miceNeuron firing
2014
Selective degeneration of a physiological subtype of spinal motor neuron in mice with SOD1-linked ALS
Hadzipasic M, Tahvildari B, Nagy M, Bian M, Horwich AL, McCormick DA. Selective degeneration of a physiological subtype of spinal motor neuron in mice with SOD1-linked ALS. Proceedings Of The National Academy Of Sciences Of The United States Of America 2014, 111: 16883-16888. PMID: 25385594, PMCID: PMC4250117, DOI: 10.1073/pnas.1419497111.Peer-Reviewed Original ResearchConceptsMN cell bodiesMotor neuronsMo of ageCell bodiesSpinal cordSpinal cord tissue slicesWhole-cell patch-clamp recordingsCell patch-clamp recordingsAdult mouse spinal cordIntrinsic electrophysiologic propertiesTwitch muscleSpinal motor neuronsLower extremity musclesTransgenic mouse modelMouse motor neuronsPatch-clamp recordingsAmyotrophic lateral sclerosisMouse spinal cordSlow-twitch muscleSteady-state firing ratePhysiological subtypesRetrograde tracingAcute slicesExtremity musclesPathophysiologic events
1991
A molecular chaperone from a thermophilic archaebacterium is related to the eukaryotic protein t-complex polypeptide-1
Trent J, Nimmesgern E, Wall J, Hartl F, Horwich A. A molecular chaperone from a thermophilic archaebacterium is related to the eukaryotic protein t-complex polypeptide-1. Nature 1991, 354: 490-493. PMID: 1836250, DOI: 10.1038/354490a0.Peer-Reviewed Original ResearchMeSH KeywordsAdenosine TriphosphatasesAmino Acid SequenceAnimalsArchaeal ProteinsBacterial ProteinsBase SequenceDNA-Binding ProteinsHeat-Shock ProteinsIntracellular Signaling Peptides and ProteinsMiceMicrotubule-Associated ProteinsMolecular ChaperonesMolecular Sequence DataNuclear ProteinsSaccharomyces cerevisiaeSequence Homology, Nucleic AcidSulfolobusT-Complex Genome RegionTemperatureUbiquitin-Protein LigasesConceptsComplex polypeptide 1Molecular chaperonesEukaryotic cytosolThermophilic archaebacteriumPolypeptide 1Ubiquitous eukaryotic proteinThermophilic factor 55Homo-oligomeric complexesMajor heat shock proteinsHeat shock proteinsChaperone componentsEukaryotic proteinsEssential proteinsProtein TAbundant proteinsSulfolobus shibataeComplex bindsS. shibataeChaperonesPrimary structureTF55ChaperoninProteinArchaebacteriaTCP1Inherited Hepatic Enzyme Defects as Candidates for Liver-Directed Gene Therapy
Horwich A. Inherited Hepatic Enzyme Defects as Candidates for Liver-Directed Gene Therapy. Current Topics In Microbiology And Immunology 1991, 168: 185-200. PMID: 1893777, DOI: 10.1007/978-3-642-76015-0_9.Peer-Reviewed Original Research
1988
Meiotic expression of human ornithine transcarbamylase in the testes of transgenic mice.
Kelley K, Chamberlain J, Nolan J, Horwich A, Kalousek F, Eisenstadt J, Herrup K, Rosenberg L. Meiotic expression of human ornithine transcarbamylase in the testes of transgenic mice. Molecular And Cellular Biology 1988, 8: 1821-1825. PMID: 2837657, PMCID: PMC363346, DOI: 10.1128/mcb.8.4.1821.Peer-Reviewed Original ResearchConceptsHuman ornithine transcarbamylaseFusion geneBase pairsProtein-coding sequencesMale germ cellsOrnithine transcarbamylaseMeiotic expressionRegulatory sequencesRegulatory regionsTransgenic micePachytene stageMouse metallothioneinTransgenic animalsGerm cellsGenesKilobasesTransgene expressionSequenceExpressionTranscarbamylaseMeiosisTetraploid
1985
Localization of DNA sequences in region Xp21 of the human X chromosome: search for molecular markers close to the Duchenne muscular dystrophy locus.
de Martinville B, Kunkel L, Bruns G, Morlé F, Koenig M, Mandel J, Horwich A, Latt S, Gusella J, Housman D. Localization of DNA sequences in region Xp21 of the human X chromosome: search for molecular markers close to the Duchenne muscular dystrophy locus. American Journal Of Human Genetics 1985, 37: 235-49. PMID: 2984924, PMCID: PMC1684559.Peer-Reviewed Original ResearchConceptsX chromosomeHuman X chromosome short armSomatic cell hybrid linesMolecular markersShort armX chromosome short armHuman X chromosomeChromosome short armDNA sequence mapGene dosage studiesOrnithine transcarbamylase geneAbnormal X chromosomeDNA sequencesDNA fragmentsHybrid linesChromosomesSequence mapsMuscular dystrophyStructural rearrangementsHuman lymphoblastsBand Xp21Duchenne muscular dystrophyGenesLociXp21
1984
Human Ornithine Transcarbamylase Locus Mapped to Band Xp21.1 Near the Duchenne Muscular Dystrophy Locus
Lindgren V, de Martinville B, Horwich A, Rosenberg L, Francke U. Human Ornithine Transcarbamylase Locus Mapped to Band Xp21.1 Near the Duchenne Muscular Dystrophy Locus. Science 1984, 226: 698-700. PMID: 6494904, DOI: 10.1126/science.6494904.Peer-Reviewed Original ResearchConceptsMitochondrial enzyme ornithine transcarbamylaseHuman ornithine transcarbamylase geneSitu hybridization experimentsOrnithine transcarbamylase geneGene mapsX chromosomeHybridization experimentsDuchenne muscular dystrophy locusShort armGenesX chromosome abnormalitiesMuscular dystrophyCell linesOrnithine transcarbamylaseLociChromosome abnormalitiesCarrier detectionChromosomesDuchenne muscular dystrophyXp21.1DNATranscarbamylaseProbeDystrophyOrnithine transcarbamylase deficiency
1983
Expression and Stabilization of Microinjected Plasmids Containing the Herpes Simplex Virus Thymidine Kinase Gene and Polyoma Virus DNA in Mouse Cells
Yamaizumi M, Horwich A, Ruddle F. Expression and Stabilization of Microinjected Plasmids Containing the Herpes Simplex Virus Thymidine Kinase Gene and Polyoma Virus DNA in Mouse Cells. Molecular And Cellular Biology 1983, 3: 511-522. PMID: 6304496, PMCID: PMC368567, DOI: 10.1128/mcb.3.4.511.Peer-Reviewed Original Research