2014
Progressive retinal degeneration and accumulation of autofluorescent lipopigments in Progranulin deficient mice
Hafler BP, Klein ZA, Zhou Z, Strittmatter SM. Progressive retinal degeneration and accumulation of autofluorescent lipopigments in Progranulin deficient mice. Brain Research 2014, 1588: 168-174. PMID: 25234724, PMCID: PMC4254024, DOI: 10.1016/j.brainres.2014.09.023.Peer-Reviewed Original ResearchMeSH KeywordsAnimalsCells, CulturedElectroretinographyGranulinsImmunohistochemistryIntercellular Signaling Peptides and ProteinsMice, Inbred C57BLMice, KnockoutMicroscopy, ConfocalNeuronal Ceroid-LipofuscinosesOptical ImagingPhotoreceptor Cells, VertebrateProgranulinsRetinal DegenerationRetinal Ganglion CellsConceptsProgranulin-deficient miceNeuronal ceroid lipofuscinosisAdult-onset neuronal ceroid lipofuscinosisDeficient miceRetinal degenerationCeroid lipofuscinosisRetinal ganglion cellsCentral nervous systemAutofluorescent storage materialMotor dysfunctionNeuropathological analysisGanglion cellsVision lossOptic atrophyEarly deathAutofluorescent lipopigmentsClinical observationsNervous systemDegenerative pathologyMiceDegenerationHomozygous mutationAutofluorescent materialPatientsNeurons
2009
Reticulon-4A (Nogo-A) Redistributes Protein Disulfide Isomerase to Protect Mice from SOD1-Dependent Amyotrophic Lateral Sclerosis
Yang YS, Harel NY, Strittmatter SM. Reticulon-4A (Nogo-A) Redistributes Protein Disulfide Isomerase to Protect Mice from SOD1-Dependent Amyotrophic Lateral Sclerosis. Journal Of Neuroscience 2009, 29: 13850-13859. PMID: 19889996, PMCID: PMC2797811, DOI: 10.1523/jneurosci.2312-09.2009.Peer-Reviewed Original ResearchMeSH KeywordsAlanineAmyotrophic Lateral SclerosisAnimalsChlorocebus aethiopsCOS CellsGlycineMaleMiceMice, CongenicMice, Inbred C57BLMice, KnockoutMice, TransgenicMolecular ChaperonesMyelin ProteinsNeuroprotective AgentsNogo ProteinsProtein Disulfide-IsomerasesSuperoxide DismutaseSuperoxide Dismutase-1Tissue DistributionConceptsAmyotrophic lateral sclerosisLateral sclerosisFatal motor neuron diseaseSubset of patientsALS disease progressionMotor neuron diseaseTransgenic mouse modelPotential therapeutic approachEndoplasmic reticulum stressHomogeneous expression patternNeuron diseaseALS pathophysiologyDisease onsetDisease progressionTherapeutic approachesMouse modelChaperone protein disulfide isomeraseReticulum stressNovel intracellular roleReticulon proteinsMiceSclerosisPatientsUnfolded protein responseNogoASerum Nogo-A levels are not elevated in amyotrophic lateral sclerosis patients
Harel NY, Cudkowicz ME, Brown RH, Strittmatter SM. Serum Nogo-A levels are not elevated in amyotrophic lateral sclerosis patients. Biomarkers 2009, 14: 414-417. PMID: 19548774, PMCID: PMC2842187, DOI: 10.1080/13547500903056051.Peer-Reviewed Original ResearchConceptsAmyotrophic lateral sclerosisALS patientsHealthy controlsAmyotrophic lateral sclerosis patientsHealthy control seraInvasive diagnostic methodsSclerosis patientsLateral sclerosisPatientsControl seraMuscle contentSerum samplesElevated levelsAdditional studiesSerumDiagnostic methodsLarge percentageSclerosisLevelsDiagnosisControlBiomarkersNogoNM
1986
Parkinson's disease: Nigral receptor changes support peptidergic role in nigrostriatal modulation
Uhl G, Hackney G, Torchia M, Stranov V, Tourtellotte W, Whitehouse P, Tran V, Strittmatter S. Parkinson's disease: Nigral receptor changes support peptidergic role in nigrostriatal modulation. Annals Of Neurology 1986, 20: 194-203. PMID: 3019228, DOI: 10.1002/ana.410200204.Peer-Reviewed Original ResearchConceptsKappa-opiate receptorsIdiopathic Parkinson's diseaseNormal human brainNigrostriatal circuitryPeptidergic influencesReceptor changesSubstantia nigraMore modest reductionsSerotonin receptorsParkinson's diseaseBenzodiazepine receptorsAutoradiographic studyModest reductionReceptorsAngiotensinSomatostatinHuman brainDiseaseDopamineBenzodiazepines IPatientsNeurotensinSubtypesBrainNigra