Cited 29 times in
Altered mitochondrial function in type 2 granular corneal dystrophy
DC Field | Value | Language |
---|---|---|
dc.contributor.author | 김응권 | - |
dc.contributor.author | 김태임 | - |
dc.contributor.author | 최승일 | - |
dc.date.accessioned | 2014-12-20T16:54:07Z | - |
dc.date.available | 2014-12-20T16:54:07Z | - |
dc.date.issued | 2011 | - |
dc.identifier.issn | 0002-9440 | - |
dc.identifier.uri | https://ir.ymlib.yonsei.ac.kr/handle/22282913/93594 | - |
dc.description.abstract | Type 2 granular corneal dystrophy (GCD2) is caused by point mutation R124H in the transforming growth factor-β-induced gene (TGFBI) and is characterized by age-dependent progression of corneal deposits. Mitochondrial features in heterozygous GCD2 and normal corneal tissues was evaluated using electron microscopy. Primary corneal fibroblasts of homozygous and normal corneas were cultured to passage 4 or 8. Keratocytes of normal corneal tissue are narrow, and details of their intracellular organelles are difficult to distinguish. Keratocytes of heterozygous GCD2 tissues exhibited many degenerative mitochondria. MitoTracker and cytochrome c staining demonstrated increased mitochondrial activity in mutated cells at early passages. Decreases in depolarized mitochondria, cellular proliferation, and expression of complexes I to V and increases in apoptotic change were observed in late-passage mutant fibroblasts. PGC-1α, ANT-1, p-Akt, and p-mTOR but not NF-κB expression demonstrated a passage-dependent decrease in all cells. Increased passage- or mutation-related intracellular reactive oxygen species and delayed proliferation of methanethiosulfonate (MTS) were recovered using application of antioxidant butylated hydroxyanisole. Mitochondrial features and function were altered in mutated GCD2 keratocytes, in particular in older cells. Alteration of mitochondrial function is critical for understanding the pathogenesis of GCD2. | - |
dc.description.statementOfResponsibility | open | - |
dc.format.extent | 684~692 | - |
dc.relation.isPartOf | AMERICAN JOURNAL OF PATHOLOGY | - |
dc.rights | CC BY-NC-ND 2.0 KR | - |
dc.rights.uri | https://creativecommons.org/licenses/by-nc-nd/2.0/kr/ | - |
dc.subject.MESH | Animals | - |
dc.subject.MESH | Corneal Dystrophies, Hereditary/genetics* | - |
dc.subject.MESH | Cytochromes c/metabolism | - |
dc.subject.MESH | Disease Progression | - |
dc.subject.MESH | Fibroblasts/metabolism | - |
dc.subject.MESH | Heterozygote | - |
dc.subject.MESH | Homozygote | - |
dc.subject.MESH | Keratinocytes/cytology | - |
dc.subject.MESH | Membrane Potentials | - |
dc.subject.MESH | Mice | - |
dc.subject.MESH | Microscopy, Electron, Transmission/methods | - |
dc.subject.MESH | Mitochondria/metabolism* | - |
dc.subject.MESH | Mitochondrial Membranes/metabolism | - |
dc.subject.MESH | Mutation | - |
dc.subject.MESH | Point Mutation | - |
dc.subject.MESH | Reactive Oxygen Species | - |
dc.subject.MESH | Transforming Growth Factor beta1/genetics | - |
dc.title | Altered mitochondrial function in type 2 granular corneal dystrophy | - |
dc.type | Article | - |
dc.contributor.college | Researcher Institutes (부설 연구소) | - |
dc.contributor.department | Corneal Dystrophy Research Institute (각막이상증연구소) | - |
dc.contributor.googleauthor | Tae-im Kim | - |
dc.contributor.googleauthor | Hanna Kim | - |
dc.contributor.googleauthor | Doo Jae Lee | - |
dc.contributor.googleauthor | Seung-Il Choi | - |
dc.contributor.googleauthor | Sang Won Kang | - |
dc.contributor.googleauthor | Eung Kweon Kim | - |
dc.identifier.doi | 10.1016/j.ajpath.2011.04.005 | - |
dc.admin.author | false | - |
dc.admin.mapping | false | - |
dc.contributor.localId | A00831 | - |
dc.contributor.localId | A01080 | - |
dc.contributor.localId | A04099 | - |
dc.relation.journalcode | J00100 | - |
dc.identifier.eissn | 1525-2191 | - |
dc.identifier.pmid | 21699880 | - |
dc.contributor.alternativeName | Kim, Eung Kweon | - |
dc.contributor.alternativeName | Kim, Tae Im | - |
dc.contributor.alternativeName | Choi, Seung Il | - |
dc.contributor.affiliatedAuthor | Kim, Eung Kweon | - |
dc.contributor.affiliatedAuthor | Kim, Tae Im | - |
dc.contributor.affiliatedAuthor | Choi, Seung Il | - |
dc.rights.accessRights | free | - |
dc.citation.volume | 179 | - |
dc.citation.number | 2 | - |
dc.citation.startPage | 684 | - |
dc.citation.endPage | 692 | - |
dc.identifier.bibliographicCitation | AMERICAN JOURNAL OF PATHOLOGY, Vol.179(2) : 684-692, 2011 | - |
dc.identifier.rimsid | 28330 | - |
dc.type.rims | ART | - |
Items in DSpace are protected by copyright, with all rights reserved, unless otherwise indicated.