Fibrates inhibit the apoptosis of Batten disease lymphoblast cells via autophagy recovery and regulation of mitochondrial membrane potential
DC Field | Value | Language |
---|---|---|
dc.contributor.author | Hong, Minho | - |
dc.contributor.author | Song, Ki Duk | - |
dc.contributor.author | Lee, Hak-Kyo | - |
dc.contributor.author | Yi, SunShin | - |
dc.contributor.author | Lee, Yong Seok | - |
dc.contributor.author | Heo, Tae-Hwe | - |
dc.contributor.author | Jun, Hyun Sik | - |
dc.contributor.author | Kim, Sung-Jo | - |
dc.date.accessioned | 2021-09-04T02:10:54Z | - |
dc.date.available | 2021-09-04T02:10:54Z | - |
dc.date.created | 2021-06-16 | - |
dc.date.issued | 2016-03 | - |
dc.identifier.issn | 1071-2690 | - |
dc.identifier.uri | https://scholar.korea.ac.kr/handle/2021.sw.korea/89364 | - |
dc.description.abstract | Batten disease (BD; also known as juvenile neuronal ceroid lipofuscinosis) is a genetic disorder inherited as an autosomal recessive trait and is characterized by blindness, seizures, cognitive decline, and early death resulting from the inherited mutation of the CLN3 gene. Mitochondrial oxidative stress, endoplasmic reticulum (ER) stress, disrupted autophagy, and enhanced apoptosis have been suggested to play a role in BD pathogenesis. Fibrates, a class of lipid-lowering drugs that induce peroxisome proliferator-activated receptor-a (PPAR-a) activation, are the most commonly used PPAR agonists. Assuming that fibrates have a neuroprotective effect, we studied the effects of fibrates, fenofibrate, bezafibrate, and gemfibrozil on apoptosis, depolarization of mitochondrial membrane, and defective autophagy in BD lymphoblast cells. The viability of fibrate-treated BD lymphoblast cells increased to levels of normal lymphoblast cells. In addition, treatment with fibrates inhibited depolarization of mitochondrial membrane potential in BD lymphoblast cells. Defective autophagy in BD lymphoblast cells was normalized when treated with fibrates as indicated by increased acridine orange staining. The recovery of autophagy in BD lymphoblast cells is most likely attributed to the upregulation of autophagy proteins, lysosomal-associated membrane protein 1 (LAMP1), and LC3 I/II, after treatment with fibrates. This study therefore suggests that fibrates may have a therapeutic potential against BD. | - |
dc.language | English | - |
dc.language.iso | en | - |
dc.publisher | SPRINGER | - |
dc.subject | PROLIFERATOR-ACTIVATED-RECEPTORS | - |
dc.subject | NEURONAL CEROID-LIPOFUSCINOSES | - |
dc.subject | LYSOSOMAL STORAGE DISORDERS | - |
dc.subject | PPAR-ALPHA AGONIST | - |
dc.subject | OXIDATIVE STRESS | - |
dc.subject | PARKINSONS-DISEASE | - |
dc.subject | MOUSE MODEL | - |
dc.subject | RAT MODEL | - |
dc.subject | FENOFIBRATE | - |
dc.subject | PROTEIN | - |
dc.title | Fibrates inhibit the apoptosis of Batten disease lymphoblast cells via autophagy recovery and regulation of mitochondrial membrane potential | - |
dc.type | Article | - |
dc.contributor.affiliatedAuthor | Jun, Hyun Sik | - |
dc.identifier.doi | 10.1007/s11626-015-9979-7 | - |
dc.identifier.scopusid | 2-s2.0-84949555128 | - |
dc.identifier.wosid | 000374350700010 | - |
dc.identifier.bibliographicCitation | IN VITRO CELLULAR & DEVELOPMENTAL BIOLOGY-ANIMAL, v.52, no.3, pp.349 - 355 | - |
dc.relation.isPartOf | IN VITRO CELLULAR & DEVELOPMENTAL BIOLOGY-ANIMAL | - |
dc.citation.title | IN VITRO CELLULAR & DEVELOPMENTAL BIOLOGY-ANIMAL | - |
dc.citation.volume | 52 | - |
dc.citation.number | 3 | - |
dc.citation.startPage | 349 | - |
dc.citation.endPage | 355 | - |
dc.type.rims | ART | - |
dc.type.docType | Article | - |
dc.description.journalClass | 1 | - |
dc.description.journalRegisteredClass | scie | - |
dc.description.journalRegisteredClass | scopus | - |
dc.relation.journalResearchArea | Cell Biology | - |
dc.relation.journalResearchArea | Developmental Biology | - |
dc.relation.journalWebOfScienceCategory | Cell Biology | - |
dc.relation.journalWebOfScienceCategory | Developmental Biology | - |
dc.subject.keywordPlus | PROLIFERATOR-ACTIVATED-RECEPTORS | - |
dc.subject.keywordPlus | NEURONAL CEROID-LIPOFUSCINOSES | - |
dc.subject.keywordPlus | LYSOSOMAL STORAGE DISORDERS | - |
dc.subject.keywordPlus | PPAR-ALPHA AGONIST | - |
dc.subject.keywordPlus | OXIDATIVE STRESS | - |
dc.subject.keywordPlus | PARKINSONS-DISEASE | - |
dc.subject.keywordPlus | MOUSE MODEL | - |
dc.subject.keywordPlus | RAT MODEL | - |
dc.subject.keywordPlus | FENOFIBRATE | - |
dc.subject.keywordPlus | PROTEIN | - |
dc.subject.keywordAuthor | Autophagy | - |
dc.subject.keywordAuthor | Mitochondria | - |
dc.subject.keywordAuthor | PPAR-alpha | - |
dc.subject.keywordAuthor | Lymphoblast | - |
dc.subject.keywordAuthor | Batten disease | - |
Items in ScholarWorks are protected by copyright, with all rights reserved, unless otherwise indicated.
(02841) 서울특별시 성북구 안암로 14502-3290-1114
COPYRIGHT © 2021 Korea University. All Rights Reserved.
Certain data included herein are derived from the © Web of Science of Clarivate Analytics. All rights reserved.
You may not copy or re-distribute this material in whole or in part without the prior written consent of Clarivate Analytics.