首页|The role of corneal endothelium in macular corneal dystrophy development and recurrence

The role of corneal endothelium in macular corneal dystrophy development and recurrence

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Macular corneal dystrophy(MCD)is a progressive,bilateral stromal dystrophic disease that arises from mutations in carbohydrate sulfo-transferase 6(CHST6).Corneal transplantation is the ultimate therapeutic solution for MCD patients.Unfortunately,postoperative recur-rence remains a significant challenge.We conducted a retrospective review of a clinical cohort comprising 102 MCD patients with 124 eyes that underwent either penetrating keratoplasty(PKP)or deep anterior lamellar keratoplasty(DALK).Our results revealed that the re-currence rate was nearly three times higher in the DALK group(39.13％,9/23 eyes)compared with the PKP group(10.89％,11/101 eyes),suggesting that surgical replacement of the corneal endothelium for treating MCD is advisable to prevent postoperative recurrence.Our experimental data confirmed the robust mRNA and protein expression of CHST6 in human corneal endothelium and the rodent homolog CHST5 in mouse endothelium.Selective knockdown of wild-type Chst5 in mouse corneal endothelium(ACsiChst5),but not in the corneal stroma,induced experimental MCD with similar extracellular matrix synthesis impairments and corneal thinning as observed in MCD patients.Mice carrying Chst5 point mutation also recapitulated clinical phenotypes of MCD,along with corneal endothelial abnormalities.Intracameral injection of wild-type Chst5 rescued the corneal impairments in ACsiChst5 mice and retarded the disease progression in Chst5 mutant mice.Overall,our study provides new mechanistic insights and therapeutic approaches for MCD treatment by highlighting the role of corneal endothelium in MCD development.

macular corneal dystrophyrecurrencecorneal endotheliumkeratan sulfatepenetrating keratoplasty

Bi-Ning Zhang、Benxiang Qi、Chunxiao Dong、Bin Zhang、Jun Cheng、Xin Wang、Suxia Li、Xiaoyun Zhuang、Shijiu Chen、Haoyun Duan、Dewei Li、Sujie Zhu、Guoyun Li、Yihai Cao、Qingjun Zhou、Lixin Xie

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Eye Institute of Shandong First Medical University,Qingdao Eye Hospital of Shandong First Medical University,Qingdao 266071,China

State Key Laboratory Cultivation Base,Shandong Provincial Key Laboratory of Ophthalmology,Qingdao 266071,China

School of Ophthalmology,Shandong First Medical University,Qingdao 250021,China

Department of Medicine,Qingdao University,Qingdao 266071,China

Eye Institute of Shandong First Medical University,Eye Hospital of Shandong First Medical University(Shandong Eye Hospital),Jinan 250021,China

Department of Ophthalmology,School of Clinical Medicine,Weifang Medical University,Weifang 261072,China

Institute of Translational Medicine,College of Medicine,Qingdao University,Qingdao 266071,China

Key Laboratory of Marine Drugs of Ministry of Education,Shandong Provincial Key Laboratory of Glycoscience and Glycotechnology,School of Medicine and Pharmacy,Ocean University of China,Qingdao 266071,China

Laboratory for Marine Drugs and Bioproducts,Pilot National Laboratory for Marine Science and Technology(Qingdao),Qingdao 266071,China

Department of Microbiology,Tumor and Cell Biology,Biomedicum,Karolinska Institutet,Stockholm 17177,Sweden

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Shandong Provincial Natural Science FoundationNational Natural Science Foundation of ChinaAcademic Promotion Program of Shandong First Medical UniversityAcademic Promotion Program of Shandong First Medical UniversityShandong Provincial Key Research and Development Program

ZR2020QH140821010912019ZL0012019RC0082021ZDSYS14

2024

10.1007/s11427-023-2364-3

中国科学:生命科学(英文版)
中国科学院

中国科学:生命科学(英文版)

CSTPCD
影响因子：0.806
ISSN：1674-7305
年,卷(期)：2024.67(2)
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