dc.contributor.author |
Yu-Wai-Man, P |
en |
dc.contributor.author |
Davies, VJ |
en |
dc.contributor.author |
Piechota, MJ |
en |
dc.contributor.author |
Cree, Lynsey |
en |
dc.contributor.author |
Votruba, M |
en |
dc.contributor.author |
Chinnery, PF |
en |
dc.coverage.spatial |
United States |
en |
dc.date.accessioned |
2011-11-16T20:02:27Z |
en |
dc.date.issued |
2009-10 |
en |
dc.identifier.citation |
Investigative Ophthalmology & Visual Science 50(10):4561-4566 Oct 2009 |
en |
dc.identifier.issn |
0146-0404 |
en |
dc.identifier.uri |
http://hdl.handle.net/2292/9097 |
en |
dc.description.abstract |
The majority of patients with autosomal dominant optic atrophy (DOA) harbor pathogenic OPA1 mutations and certain missense mutations, mostly within the GTPase domain, have recently been shown to cause multiple mitochondrial DNA (mtDNA) deletions in skeletal muscle. This raises the possibility that the optic neuropathy could be the result of secondary mtDNA defects accumulating within retinal ganglion cells (RGCs). To explore this hypothesis, the authors looked for evidence of mitochondrial dysfunction in a mouse model of DOA and documented the visual and neurologic progression in aging mutant mice. |
en |
dc.language |
eng |
en |
dc.publisher |
Association for research in vision and ophthalmology |
en |
dc.relation.ispartofseries |
Investigative Ophthalmology & Visual Science |
en |
dc.rights |
Items in ResearchSpace are protected by copyright, with all rights reserved, unless otherwise indicated. Previously published items are made available in accordance with the copyright policy of the publisher. Details obtained from http://www.sherpa.ac.uk/romeo/issn/0146-0404/ |
en |
dc.rights.uri |
https://researchspace.auckland.ac.nz/docs/uoa-docs/rights.htm |
en |
dc.subject |
Adenosine Triphosphatases |
en |
dc.subject |
Animals |
en |
dc.subject |
Cytochrome-c Oxidase Deficiency |
en |
dc.subject |
DNA, Mitochondrial |
en |
dc.subject |
Disease Models, Animal |
en |
dc.subject |
Electron Transport Complex IV |
en |
dc.subject |
GTP Phosphohydrolases |
en |
dc.subject |
Gene Deletion |
en |
dc.subject |
Mice |
en |
dc.subject |
Mice, Inbred C57BL |
en |
dc.subject |
Mitochondria, Muscle |
en |
dc.subject |
Mitochondrial Diseases |
en |
dc.subject |
Muscle, Skeletal |
en |
dc.subject |
Mutation, Missense |
en |
dc.subject |
Nystagmus, Optokinetic |
en |
dc.subject |
Optic Atrophy, Autosomal Dominant |
en |
dc.subject |
Retinal Ganglion Cells |
en |
dc.subject |
Succinate Dehydrogenase |
en |
dc.subject |
Vision Disorders |
en |
dc.title |
Secondary mtDNA defects do not cause optic nerve dysfunction in a mouse model of dominant optic atrophy. |
en |
dc.type |
Journal Article |
en |
dc.identifier.doi |
10.1167/iovs.09-3634 |
en |
pubs.issue |
10 |
en |
pubs.begin-page |
4561 |
en |
pubs.volume |
50 |
en |
dc.rights.holder |
Copyright: Association for Research in Vision and Ophthalmology |
en |
dc.identifier.pmid |
19443720 |
en |
pubs.end-page |
4566 |
en |
dc.rights.accessrights |
http://purl.org/eprint/accessRights/RestrictedAccess |
en |
pubs.subtype |
Article |
en |
pubs.elements-id |
211196 |
en |
pubs.org-id |
Medical and Health Sciences |
en |
pubs.org-id |
School of Medicine |
en |
pubs.org-id |
Obstetrics and Gynaecology |
en |
dc.identifier.eissn |
1552-5783 |
en |
dc.identifier.pii |
iovs.09-3634 |
en |
pubs.record-created-at-source-date |
2011-11-17 |
en |
pubs.dimensions-id |
19443720 |
en |