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Autophagy in granular corneal dystrophy type 2

Authors
 Seung-Il Choi  ;  Eung Kweon Kim 
Citation
 Experimental Eye Research, Vol.144 : 14-21, 2016 
Journal Title
 Experimental Eye Research 
ISSN
 0014-4835 
Issue Date
2016
MeSH
Autophagy/physiology* ; Corneal Dystrophies, Hereditary/etiology ; Corneal Dystrophies, Hereditary/metabolism* ; Extracellular Matrix Proteins/genetics ; Extracellular Matrix Proteins/metabolism ; Humans ; Lysosomes ; Proteolysis ; Transforming Growth Factor beta/genetics ; Transforming Growth Factor beta/metabolism ; Substances ; Extracellular Matrix Proteins ; Transforming Growth Factor beta ; betaIG-H3 protein
Keywords
Autophagy ; Corneal fibroblasts ; ECM endocytosis ; GCD2 ; TGFBIp
Abstract
Autophagy is a lysosomal degradative process that is essential for cellular homeostasis and metabolic stress adaptation. Defective autophagy is involved in the pathogenesis of many diseases including granular corneal dystrophy type 2 (GCD2). GCD2 is an autosomal dominant disorder caused by substitution of histidine for arginine at codon 124 (R124H) in the transforming growth factor β-induced gene (TGFBI) on chromosome 5q31. Transforming growth factor β-induced protein (TGFBIp) is degraded by autophagy, but mutant-TGFBIp accumulates in autophagosomes and/or lysosomes, despite significant activation of basal autophagy, in GCD2 corneal fibroblasts. Furthermore, inhibition of autophagy induces cell death of GCD2 corneal fibroblasts through active caspase-3. As there is currently no pharmacological treatment for GCD2, development of novel therapies is required. A potential strategy for preventing cytoplasmic accumulation of mutant-TGFBIp in GCD2 corneal fibroblasts is to enhance mutant-TGFBIp degradation. This could be achieved by activation of the autophagic pathway. Here, we will consider the role and the potential therapeutic benefits of autophagy in GCD2, with focus on TGFBIp degradation, in light of the recently established role of autophagy in protein degradation.
Full Text
http://www.sciencedirect.com/science/article/pii/S0014483515300294
DOI
10.1016/j.exer.2015.09.008
Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Ophthalmology (안과학교실) > 1. Journal Papers
5. Research Institutes (연구소) > Corneal Dystrophy Research Institute (각막이상증연구소) > 1. Journal Papers
Yonsei Authors
김응권(Kim, Eung Kweon) ORCID logo https://orcid.org/0000-0002-1453-8042
최승일(Choi, Seung Il) ORCID logo https://orcid.org/0000-0001-7168-8795
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URI
https://ir.ymlib.yonsei.ac.kr/handle/22282913/146422
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