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dc.contributor.authorWali, Gautam
dc.contributor.authorSue, Carolyn M
dc.contributor.authorMackay-Sim, Alan
dc.date.accessioned2019-08-20T04:37:10Z
dc.date.available2019-08-20T04:37:10Z
dc.date.issued2018
dc.identifier.issn2076-3425
dc.identifier.doi10.3390/brainsci8080142
dc.identifier.urihttp://hdl.handle.net/10072/383277
dc.description.abstractHereditary spastic paraplegia is an inherited, progressive paralysis of the lower limbs first described by Adolph Strümpell in 1883 with a further detailed description of the disease by Maurice Lorrain in 1888. Today, more than 100 years after the first case of HSP was described, we still do not know how mutations in HSP genes lead to degeneration of the corticospinal motor neurons. This review describes how patient-derived stem cells contribute to understanding the disease mechanism at the cellular level and use this for discovery of potential new therapeutics, focusing on SPAST mutations, the most common cause of HSP.
dc.description.peerreviewedYes
dc.languageEnglish
dc.language.isoeng
dc.publisherMultidisciplinary Digital Publishing Institute (MDPI)
dc.relation.ispartofissue8
dc.relation.ispartofjournalBrain Sciences
dc.relation.ispartofvolume8
dc.subject.fieldofresearchNeurosciences
dc.subject.fieldofresearchPsychology
dc.subject.fieldofresearchCognitive Sciences
dc.subject.fieldofresearchcode1109
dc.subject.fieldofresearchcode1701
dc.subject.fieldofresearchcode1702
dc.titlePatient-Derived Stem Cell Models in SPAST HSP: Disease Modelling and Drug Discovery
dc.typeJournal article
dc.type.descriptionC1 - Articles
dc.type.codeC - Journal Articles
dcterms.licensehttp://creativecommons.org/licenses/by/4.0/
dc.description.versionVersion of Record (VoR)
gro.rights.copyright© 2018 by the authors. Licensee MDPI, Basel, Switzerland. This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited (CC BY 4.0).
gro.hasfulltextFull Text
gro.griffith.authorMackay-Sim, Alan


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