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AuthorSemelink-Sedlacek, Lena Luisadc.contributor.author
Date of accession2020-06-29T13:58:26Zdc.date.accessioned
Available in OPARU since2020-06-29T13:58:26Zdc.date.available
Year of creation2016dc.date.created
Date of first publication2020-06-29dc.date.issued
AbstractAmyotrophic lateral sclerosis (ALS) is the most common motoneuron disease, which is characterized by adult onset of a rapidly progression of the degeneration of the upper and lower motoneurons (MNs). The hexanucleotide repeat expansion of chromosome nine open reading frame 72 (C9ORF72) is the most common mutation in familial and sporadic ALS cases, but the mutation has been sparsely characterised. In vitro cell models of primary rodent MNs or secondary cell systems and some animal models as well as postmortem brain tissue have hitherto been the main model systems in C9-ALS research. However, these model systems are widely restricted in their capacity to capture the full range of human ALS pathogenesis, given the present technical difficulty in the artificial induction of huge repeat expansions. Therefore, we established stem cell-based model systems to study the pathogenesis of ALS in a human-derived, patient-specific setting.dc.description.abstract
Languageendc.language.iso
PublisherUniversität Ulmdc.publisher
LicenseStandarddc.rights
Link to license texthttps://oparu.uni-ulm.de/xmlui/license_v3dc.rights.uri
Dewey Decimal GroupDDC 570 / Life sciencesdc.subject.ddc
Dewey Decimal GroupDDC 610 / Medicine & healthdc.subject.ddc
MeSHAmyotrophic lateral sclerosisdc.subject.mesh
MeSHC9orf72 proteindc.subject.mesh
MeSHNeural stem cellsdc.subject.mesh
TitleModeling C9ORF72 pathomechanisms in patient-derived cellsdc.title
Resource typeDissertationdc.type
Date of acceptance2020-06-25dcterms.dateAccepted
RefereeBöckers, Tobias M.dc.contributor.referee
RefereeOtto, Markusdc.contributor.referee
DOIhttp://dx.doi.org/10.18725/OPARU-32206dc.identifier.doi
PPN1702946045dc.identifier.ppn
URNhttp://nbn-resolving.de/urn:nbn:de:bsz:289-oparu-32268-8dc.identifier.urn
GNDMyatrophische Lateralsklerosedc.subject.gnd
GNDStammzelledc.subject.gnd
FacultyMedizinische Fakultätuulm.affiliationGeneral
InstitutionInstitut für Anatomie und Zellbiologieuulm.affiliationSpecific
InstitutionUKU. Klinik für Neurologieuulm.affiliationSpecific
Grantor of degreeMedizinische Fakultätuulm.thesisGrantor
DCMI TypeTextuulm.typeDCMI
CategoryPublikationenuulm.category
University Bibliographyjauulm.unibibliographie


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