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

作     者：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 

作者机构：Eye Institute of Shandong First Medical UniversityQingdao Eye Hospital of Shandong First Medical UniversityQingdao 266071China State Key Laboratory Cultivation BaseShandong Provincial Key Laboratory of OphthalmologyQingdao 266071China School of OphthalmologyShandong First Medical UniversityQingdao 250021China Department of MedicineQingdao UniversityQingdao 266071China Eye Institute of Shandong First Medical UniversityEye Hospital of Shandong First Medical University(Shandong Eye Hospital)Jinan 250021China Department of OphthalmologySchool of Clinical MedicineWeifang Medical UniversityWeifang 261072China Institute of Translational MedicineCollege of MedicineQingdao UniversityQingdao 266071China Key Laboratory of Marine Drugs of Ministry of EducationShandong Provincial Key Laboratory of Glycoscience and GlycotechnologySchool of Medicine and PharmacyOcean University of ChinaQingdao 266071China Laboratory for Marine Drugs and BioproductsPilot National Laboratory for Marine Science and Technology(Qingdao)Qingdao 266071China Department of MicrobiologyTumor and Cell BiologyBiomedicumKarolinska InstitutetStockholm 17177Sweden 

出 版 物：《Science China(Life Sciences)》 (中国科学（生命科学英文版）)

年 卷 期：2024年第67卷第2期

页      面：332-344页

核心收录：

学科分类：0710[理学-生物学] 1002[医学-临床医学] 100212[医学-眼科学] 10[医学] 

基　　金：supported by the Shandong Provincial Natural Science Foundation(ZR2020QH140) the National Natural Science Foundation of China(82101091) the Academic Promotion Program of Shandong First Medical University(2019ZL001,2019RC008) the Shandong Provincial Key Research and Development Program(2021ZDSYS14) 

主　　题：macular corneal dystrophy recurrence corneal endothelium keratan sulfate penetrating keratoplasty 

摘      要：Macular corneal dystrophy(MCD)is a progressive,bilateral stromal dystrophic disease that arises from mutations in carbohydrate sulfotransferase 6(CHST6).Corneal transplantation is the ultimate therapeutic solution for MCD ***,postoperative recurrence remains a significant *** 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 recurrence 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 *** experimental data confirmed the robust m RNA and protein expression of CHST6 in human corneal endothelium and the rodent homolog CHST5 in mouse *** knockdown of wild-type Chst5 in mouse corneal endothelium(AC^(siChst5)),but not in the corneal stroma,induced experimental MCD with similar extracellular matrix synthesis impairments and corneal thinning as observed in MCD *** carrying Chst5 point mutation also recapitulated clinical phenotypes of MCD,along with corneal endothelial *** injection of wild-type Chst5 rescued the corneal impairments in AC^(siChst5)mice and retarded the disease progression in Chst5 mutant ***,our study provides new mechanistic insights and therapeutic approaches for MCD treatment by highlighting the role of corneal endothelium in MCD development.