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Comparative Proteome Research in a Zebrafish Model for Vanishing White Matter Disease

Authors
 Doeun Kim  ;  Yu-Ri Lee  ;  Tae-Ik Choi  ;  Se-Hee Kim  ;  Hoon-Chul Kang  ;  Cheol-Hee Kim  ;  Sangkyu Lee 
Citation
 INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES, Vol.22(5) : 2707, 2021-03 
Journal Title
 INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES 
Issue Date
2021-03
Keywords
EIF2B ; SLC1A4 ; Vanishing White Matter disease ; comparative proteomics
Abstract
Vanishing white matter (VWM) disease is a genetic leukodystrophy leading to severe neurological disease and early death. VWM is caused by bi-allelic mutations in any of the five genes encoding the subunits of the eukaryotic translation factor 2B (EIF2B). Previous studies have attempted to investigate the molecular mechanism of VWN by constructing models for each subunit of EIF2B that causes VWM disease. The underlying molecular mechanisms of the way in which mutations in EIF2B3 result in VWM are largely unknown. Based on our recent results, we generated an eif2b3 knockout (eif2b3-/-) zebrafish model and performed quantitative proteomic analysis between the wild-type (WT) and eif2b3-/- zebrafish, and identified 25 differentially expressed proteins. Four proteins were significantly upregulated, and 21 proteins were significantly downregulated in eif2b3-/- zebrafish compared to WT. Lon protease and the neutral amino acid transporter SLC1A4 were significantly increased in eif2b3-/- zebrafish, and crystallin proteins were significantly decreased. The differential expression of proteins was confirmed by the evaluation of mRNA levels in eif2b3-/- zebrafish, using whole-mount in situ hybridization analysis. This study identified proteins which candidates as key regulators of the progression of VWN disease, using quantitative proteomic analysis in the first EIF2B3 animal model of VWN disease.
Files in This Item:
T202100890.pdf Download
DOI
10.3390/ijms22052707
Appears in Collections:
1. College of Medicine (의과대학) > Others (기타) > 1. Journal Papers
Yonsei Authors
Kang, Hoon Chul(강훈철) ORCID logo https://orcid.org/0000-0002-3659-8847
Kim, Se Hee(김세희) ORCID logo https://orcid.org/0000-0001-7773-1942
URI
https://ir.ymlib.yonsei.ac.kr/handle/22282913/182278
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