2D DIGE proteomic analysis highlights delayed postnatal repression of α‐fetoprotein expression in homocystinuria model mice

Cystathionine β‐synthase‐deficient (Cbs−/−) mice, an animal model for homocystinuria, exhibit hepatic steatosis and juvenile semilethality via as yet unknown mechanisms. The plasma protein profile ofCbs−/− mice was investigated by proteomic analysis using two‐dimensional difference gel electrophores...

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Main Authors: Shotaro Kamata, Noriyuki Akahoshi, Isao Ishii
Format: Article
Language:English
Published: Wiley 2015-01-01
Series:FEBS Open Bio
Subjects:
Online Access:https://doi.org/10.1016/j.fob.2015.06.008
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author Shotaro Kamata
Noriyuki Akahoshi
Isao Ishii
author_facet Shotaro Kamata
Noriyuki Akahoshi
Isao Ishii
author_sort Shotaro Kamata
collection DOAJ
description Cystathionine β‐synthase‐deficient (Cbs−/−) mice, an animal model for homocystinuria, exhibit hepatic steatosis and juvenile semilethality via as yet unknown mechanisms. The plasma protein profile ofCbs−/− mice was investigated by proteomic analysis using two‐dimensional difference gel electrophoresis and matrix‐assisted laser desorption/ionization‐time of flight/mass spectrometry. We found hyperaccumulation of α‐fetoprotein (AFP) and downregulation of most other plasma proteins. AFP was highly expressed in fetal liver, but its expression declined dramatically via transcriptional repression after birth in both wild‐type andCbs−/− mice. However, the repression was delayed inCbs−/− mice, causing high postnatal AFP levels, which may relate to transcriptional repression of most plasma proteins originating from liver and the observed hepatic dysfunction.
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spelling doaj.art-4fb54efd06fa427cbfa40306e152f7d42022-12-22T04:22:07ZengWileyFEBS Open Bio2211-54632015-01-015153554110.1016/j.fob.2015.06.0082D DIGE proteomic analysis highlights delayed postnatal repression of α‐fetoprotein expression in homocystinuria model miceShotaro Kamata0Noriyuki Akahoshi1Isao Ishii2Department of Biochemistry, Keio University Graduate School of Pharmaceutical Sciences, Tokyo 105-8512, JapanDepartment of Immunology, Akita University Graduate School of Medicine, Akita 010-8543, JapanDepartment of Biochemistry, Keio University Graduate School of Pharmaceutical Sciences, Tokyo 105-8512, JapanCystathionine β‐synthase‐deficient (Cbs−/−) mice, an animal model for homocystinuria, exhibit hepatic steatosis and juvenile semilethality via as yet unknown mechanisms. The plasma protein profile ofCbs−/− mice was investigated by proteomic analysis using two‐dimensional difference gel electrophoresis and matrix‐assisted laser desorption/ionization‐time of flight/mass spectrometry. We found hyperaccumulation of α‐fetoprotein (AFP) and downregulation of most other plasma proteins. AFP was highly expressed in fetal liver, but its expression declined dramatically via transcriptional repression after birth in both wild‐type andCbs−/− mice. However, the repression was delayed inCbs−/− mice, causing high postnatal AFP levels, which may relate to transcriptional repression of most plasma proteins originating from liver and the observed hepatic dysfunction.https://doi.org/10.1016/j.fob.2015.06.008α-FetoproteinCystathionine β-synthaseMALDI-TOF/MSProteomicsTranscriptional regulation2D DIGE
spellingShingle Shotaro Kamata
Noriyuki Akahoshi
Isao Ishii
2D DIGE proteomic analysis highlights delayed postnatal repression of α‐fetoprotein expression in homocystinuria model mice
FEBS Open Bio
α-Fetoprotein
Cystathionine β-synthase
MALDI-TOF/MS
Proteomics
Transcriptional regulation
2D DIGE
title 2D DIGE proteomic analysis highlights delayed postnatal repression of α‐fetoprotein expression in homocystinuria model mice
title_full 2D DIGE proteomic analysis highlights delayed postnatal repression of α‐fetoprotein expression in homocystinuria model mice
title_fullStr 2D DIGE proteomic analysis highlights delayed postnatal repression of α‐fetoprotein expression in homocystinuria model mice
title_full_unstemmed 2D DIGE proteomic analysis highlights delayed postnatal repression of α‐fetoprotein expression in homocystinuria model mice
title_short 2D DIGE proteomic analysis highlights delayed postnatal repression of α‐fetoprotein expression in homocystinuria model mice
title_sort 2d dige proteomic analysis highlights delayed postnatal repression of α fetoprotein expression in homocystinuria model mice
topic α-Fetoprotein
Cystathionine β-synthase
MALDI-TOF/MS
Proteomics
Transcriptional regulation
2D DIGE
url https://doi.org/10.1016/j.fob.2015.06.008
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