Cited 73 times in
Pathogenesis and treatments of TGFBI corneal dystrophies
DC Field | Value | Language |
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dc.contributor.author | 김응권 | - |
dc.contributor.author | 김태임 | - |
dc.contributor.author | 맹용선 | - |
dc.contributor.author | 지용우 | - |
dc.contributor.author | 최승일 | - |
dc.date.accessioned | 2017-02-24T03:13:21Z | - |
dc.date.available | 2017-02-24T03:13:21Z | - |
dc.date.issued | 2016 | - |
dc.identifier.issn | 1350-9462 | - |
dc.identifier.uri | https://ir.ymlib.yonsei.ac.kr/handle/22282913/146281 | - |
dc.description.abstract | Transforming growth factor beta-induced (TGFBI) corneal dystrophies are a group of inherited progressive corneal diseases. Accumulation of transforming growth factor beta-induced protein (TGFBIp) is involved in the pathogenesis of TGFBI corneal dystrophies; however, the exact molecular mechanisms are not fully elucidated. In this review article, we summarize the current knowledge of TGFBI corneal dystrophies including clinical manifestations, epidemiology, most common and recently reported associated mutations for each disease, and treatment modalities. We review our current understanding of the molecular mechanisms of granular corneal dystrophy type 2 (GCD2) and studies of other TGFBI corneal dystrophies. In GCD2 corneal fibroblasts, alterations of morphological characteristics of corneal fibroblasts, increased susceptibility to intracellular oxidative stress, dysfunctional and fragmented mitochondria, defective autophagy, and alterations of cell cycle were observed. Other studies of mutated TGFBIp show changes in conformational structure, stability and proteolytic properties in lattice and granular corneal dystrophies. Future research should be directed toward elucidation of the biochemical mechanism of deposit formation, the relationship between the mutated TGFBIp and the other materials in the extracellular matrix, and the development of gene therapy and pharmaceutical agents. | - |
dc.description.statementOfResponsibility | restriction | - |
dc.format.extent | 67~88 | - |
dc.language | English | - |
dc.publisher | Pergamon | - |
dc.relation.isPartOf | PROGRESS IN RETINAL AND EYE RESEARCH | - |
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 | Autophagy/physiology | - |
dc.subject.MESH | Cornea/metabolism | - |
dc.subject.MESH | Corneal Dystrophies, Hereditary*/etiology | - |
dc.subject.MESH | Corneal Dystrophies, Hereditary*/genetics | - |
dc.subject.MESH | Corneal Dystrophies, Hereditary*/metabolism | - |
dc.subject.MESH | Corneal Dystrophies, Hereditary*/therapy | - |
dc.subject.MESH | Fibroblasts/pathology | - |
dc.subject.MESH | Genetic Predisposition to Disease | - |
dc.subject.MESH | Humans | - |
dc.subject.MESH | Mutation | - |
dc.subject.MESH | Oxidative Stress/physiology | - |
dc.subject.MESH | Transforming Growth Factor beta/metabolism* | - |
dc.title | Pathogenesis and treatments of TGFBI corneal dystrophies | - |
dc.type | Article | - |
dc.publisher.location | England | - |
dc.contributor.college | College of Medicine | - |
dc.contributor.department | Dept. of Ophthalmology | - |
dc.contributor.googleauthor | Kyung Eun Han | - |
dc.contributor.googleauthor | Seung-il Choi | - |
dc.contributor.googleauthor | Tae-im Kim | - |
dc.contributor.googleauthor | Yong-Sun Maeng | - |
dc.contributor.googleauthor | R. Doyle Stulting | - |
dc.contributor.googleauthor | Yong Woo Ji | - |
dc.contributor.googleauthor | Eung Kweon Kim | - |
dc.identifier.doi | 10.1016/j.preteyeres.2015.11.002 | - |
dc.contributor.localId | A00831 | - |
dc.contributor.localId | A01080 | - |
dc.contributor.localId | A01346 | - |
dc.contributor.localId | A03967 | - |
dc.contributor.localId | A04099 | - |
dc.relation.journalcode | J02895 | - |
dc.identifier.eissn | 1873-1635 | - |
dc.identifier.pmid | 26612778 | - |
dc.identifier.url | http://www.sciencedirect.com/science/article/pii/S1350946215300021 | - |
dc.subject.keyword | Genetics | - |
dc.subject.keyword | Granular corneal dystrophy type 2 | - |
dc.subject.keyword | Molecular mechanism | - |
dc.subject.keyword | Transforming growth factor beta-induced corneal dystrophies | - |
dc.subject.keyword | Transforming growth factor beta-induced protein | - |
dc.contributor.alternativeName | Kim, Eung Kweon | - |
dc.contributor.alternativeName | Kim, Tae Im | - |
dc.contributor.alternativeName | Maeng, Yong Sun | - |
dc.contributor.alternativeName | Ji, Yong Woo | - |
dc.contributor.alternativeName | Choi, Seung Il | - |
dc.contributor.affiliatedAuthor | Kim, Eung Kweon | - |
dc.contributor.affiliatedAuthor | Kim, Tae Im | - |
dc.contributor.affiliatedAuthor | Maeng, Yong Sun | - |
dc.contributor.affiliatedAuthor | Ji, Yong Woo | - |
dc.contributor.affiliatedAuthor | Choi, Seung Il | - |
dc.citation.volume | 50 | - |
dc.citation.startPage | 67 | - |
dc.citation.endPage | 88 | - |
dc.identifier.bibliographicCitation | PROGRESS IN RETINAL AND EYE RESEARCH, Vol.50 : 67-88, 2016 | - |
dc.date.modified | 2017-02-24 | - |
dc.identifier.rimsid | 51354 | - |
dc.type.rims | ART | - |
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