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dc.contributor.authorErickson, Robert
dc.date.accessioned2019-11-14T19:38:40Z
dc.date.available2019-11-14T19:38:40Z
dc.date.issued2019-08
dc.identifier.citationJ. Applied Genet. 60:357-365.en_US
dc.identifier.issn1234-1983
dc.identifier.pmid31485950
dc.identifier.doi10.1007/s13353-019-00517-8
dc.identifier.urihttp://hdl.handle.net/10150/635745
dc.description.abstractThe Npc1(nih/nih)-null model and the Npc1(nmf164/nmf164) hypomorph models of Niemann-Pick C1 (NPC1) disease show defects in olfaction. We have tested the effects of the life-prolonging treatment hydroxypropyl-beta-cyclodextrin (HPBCD) on olfaction and neural stem cell numbers when delivered either systemically or by nasal inhalation. Using the paradigm of finding a hidden cube of food after overnight food deprivation, Npc1(nih/nih) homozygous mice showed a highly significant delay in finding the food compared with wild-type mice. Npc1(nmf164/nmf164) homozygous mice showed an early loss of olfaction which was mildly corrected by somatic delivery of HPBCD which also increased the number of neural stem cells in the mutant but did not change the number in wild-type mice. In contrast, nasal delivery of this drug, at 1/5 the dosage used for somatic delivery, to Npc1(nmf164/nmf164) mutant mice delayed loss of olfaction but the control of nasal delivered saline did so as well. The nasal delivery of HPBCD to wild-type mice caused loss of olfaction but nasal delivery of saline did not. Neural stem cell counts were not improved by nasal therapy with HPBCD. We credit the delay in olfaction found with the treatment, a delay which was also found for time of death, to a large amount of stimulation the mice received with handling during the nasal delivery.en_US
dc.description.sponsorshipTelethon Foundation - Italy, Fondazione Telethon [GGP13183]; Ateneo La Sapienza, Sapienza University Rome [RM11615501ED6577]en_US
dc.language.isoenen_US
dc.publisherSPRINGER HEIDELBERGen_US
dc.rights© Institute of Plant Genetics, Polish Academy of Sciences, Poznan 2019.en_US
dc.rights.urihttp://rightsstatements.org/vocab/InC/1.0/
dc.subjectOlfactionen_US
dc.subjectNiemann-Pick C1 diseaseen_US
dc.subjectHydroxypropyl-beta-cyclodextrinsen_US
dc.subjectNeural stem cellsen_US
dc.subjectNasal deliveryen_US
dc.titleThe effects of hydroxypropyl-beta-cyclodextrin on olfaction in mouse models of Niemann-Pick C1 Diseaseen_US
dc.typeArticleen_US
dc.contributor.departmentUniv Arizona, Sch Med, Dept Pediaten_US
dc.identifier.journalJOURNAL OF APPLIED GENETICSen_US
dc.description.note12 month embargo; first Online: 04 September 2019en_US
dc.description.collectioninformationThis item from the UA Faculty Publications collection is made available by the University of Arizona with support from the University of Arizona Libraries. If you have questions, please contact us at repository@u.library.arizona.edu.en_US
dc.eprint.versionFinal accepted manuscripten_US


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