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Altered mitochondrial function in type 2 granular corneal dystrophy

Authors
 Tae-im Kim  ;  Hanna Kim  ;  Doo Jae Lee  ;  Seung-Il Choi  ;  Sang Won Kang  ;  Eung Kweon Kim 
Citation
 American Journal of Pathology, Vol.179(2) : 684-692, 2011 
Journal Title
 American Journal of Pathology 
ISSN
 0002-9440 
Issue Date
2011
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.
URI
https://ir.ymlib.yonsei.ac.kr/handle/22282913/93594
DOI
10.1016/j.ajpath.2011.04.005
Appears in Collections:
1. Journal Papers (연구논문) > 5. Research Institutes (연구소) > Corneal Dystrophy Research Institute (각막이상증연구소)
1. Journal Papers (연구논문) > 1. College of Medicine (의과대학) > Dept. of Ophthalmology (안과학교실)
Yonsei Authors
김응권(Kim, Eung Kweon) ; 김태임(Kim, Tae Im) ; 최승일(Choi, Seung Il)
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