0000000000548625

AUTHOR

P. Vogel

showing 4 related works from this author

Hereditary motor sensory neuropathy type II with neurofilament accumulation: new finding or new disorder?

1985

Peroneal muscular atrophy is now known to be heterogeneous and to be due to various underlying genetic mechanisms. Exploring this heterogeneity further, we report on a German kinship with the clinical, genetic, and nerve conduction features of hereditary motor and sensory neuropathy type II (HMSN type II) but whose sural nerves on biopsy were found to show infrequent axonal swellings with neurofilament accumulations not previously described. The dominant inheritance and absence of kinky hair set this disorder apart from giant axonal neuropathy. There was no history of toxic exposure to industrial chemicals. We conclude that the disorder either is a new type of HMSN or is HMSN type II with p…

AdultMaleNeurofilamentAdolescentBiopsyCardiomyopathyNeural ConductionCardiovascular System03 medical and health sciences0302 clinical medicineSural NerveEvoked Potentials SomatosensoryBiopsymedicineHumansAxonHereditary Sensory and Autonomic NeuropathiesChildCytoskeleton030304 developmental biologyGiant axonal neuropathyAged0303 health sciencesmedicine.diagnostic_testbusiness.industryElectromyographyPeroneal muscular atrophyMiddle Agedmedicine.diseaseAxonsPedigreeMicroscopy ElectronMuscular Atrophymedicine.anatomical_structureNeurologyEvoked Potentials VisualFemaleNeurology (clinical)AbnormalityHereditary motor and sensory neuropathybusinessNeuroscience030217 neurology & neurosurgeryAnnals of neurology
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Neuropathologic and morphometric studies in hereditary motor and sensory neuropathy type II with neurofilament accumulation.

1986

Histological, electron microscopic and morphometric data on sural nerve, muscle, and skin biopsies of three patients affected by autosomal dominant hereditary motor and sensory neuropathy type II with neurofilament accumulation, whose neurological, cardiological and electrophysiological data have been provided in a previous paper disclosed focally enlarged myelinated axons, due to aggregation of neurofilaments in sural nerves of all 3 biopsied patients, as well as densely packed clusters of filaments in occasional non-myelinated axons without axonal enlargement, in several fibroblasts and endothelial cells in muscle and particularly in skin. This accumulation of filaments was less pronounce…

Pathologymedicine.medical_specialtyNeurofilamentNeurologyIntermediate FilamentsSural nerveDermatologyBiologylaw.invention03 medical and health sciences0302 clinical medicineSural NervelawCharcot-Marie-Tooth DiseasemedicineHumansHereditary Sensory and Autonomic NeuropathiesIntermediate filamentCytoskeletonMyelin Sheath030304 developmental biologySkinMotor Neurons0303 health sciencesGeneral NeuroscienceMusclesGeneral MedicineAnatomymedicine.diseaseAxonsPsychiatry and Mental healthElectrophysiologyMicroscopy ElectronMuscular Atrophynervous systemUltrastructureNeurology (clinical)Electron microscopeHereditary motor and sensory neuropathy030217 neurology & neurosurgeryItalian journal of neurological sciences
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Phylogeographical footprints of the Strait of Gibraltar and Quaternary climatic fluctuations in the western Mediterranean: a case study with the grea…

2005

Correspondance: cosson@supagro.inra.fr; International audience; We used mitochondrial cyt b sequences to investigate the phylogenetic relationships of Crocidura russula (sensu lato) populations across the Strait of Gibraltar, western Europe, Maghreb, and the Mediterranean and Atlantic islands. This revealed very low genetic divergence between European and Moroccan populations. The application of a molecular clock previously calibrated for shrews suggested that the separation of European from Moroccan lineages occurred less than 60 000 bp, which is at least 5 million years (Myr) after the reopening of the Strait of Gibraltar. This means that an overwater dispersal event was responsible for t…

[SDV.EE]Life Sciences [q-bio]/Ecology environmentTunisiaGeographymtDNAQuaternary historyClimateShrewsGenetic VariationSequence Analysis DNA[SDV.BID]Life Sciences [q-bio]/BiodiversityCytochromes bphylogeographyNorth AfricaEuropehuman-assisted dispersalMoroccoHaplotypesspeciationMediterranean SeaAnimalsEurope human-assisted dispersal mtDNA North Africa phylogeography Quaternary history speciation[SDE.BE]Environmental Sciences/Biodiversity and EcologySequence AlignmentPhylogeny
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Measurement of the Spectral Shape of the beta-decay of 137Xe to the Ground State of 137Cs in EXO-200 and Comparison with Theory

2020

We report on a comparison between the theoretically predicted and experimentally measured spectra of the first-forbidden non-unique $\beta$-decay transition $^{137}\textrm{Xe}(7/2^-)\to\,^{137}\textrm{Cs}(7/2^+)$. The experimental data were acquired by the EXO-200 experiment during a deployment of an AmBe neutron source. The ultra-low background environment of EXO-200, together with dedicated source deployment and analysis procedures, allowed for collection of a pure sample of the decays, with an estimated signal-to-background ratio of more than 99-to-1 in the energy range from 1075 to 4175 keV. In addition to providing a rare and accurate measurement of the first-forbidden non-unique $\bet…

Nuclear Theory (nucl-th)Nuclear TheoryFOS: Physical sciencesNuclear Experiment (nucl-ex)Nuclear Experiment
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