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

Shotaro Kamata, Noriyuki Akahoshi, Isao Ishii

Research output: Contribution to journalArticle

3 Citations (Scopus)


Cystathionine β-synthase-deficient (Cbs<sup>-/-</sup>) mice, an animal model for homocystinuria, exhibit hepatic steatosis and juvenile semilethality via as yet unknown mechanisms. The plasma protein profile of Cbs<sup>-/-</sup> 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 and Cbs<sup>-/-</sup> mice. However, the repression was delayed in Cbs<sup>-/-</sup> mice, causing high postnatal AFP levels, which may relate to transcriptional repression of most plasma proteins originating from liver and the observed hepatic dysfunction.

Original languageEnglish
Pages (from-to)535-541
Number of pages7
JournalFEBS Open Bio
Publication statusPublished - 2015
Externally publishedYes



  • 2D DIGE
  • Cystathionine β-synthase
  • Proteomics
  • Transcriptional regulation
  • α-Fetoprotein

ASJC Scopus subject areas

  • Biochemistry, Genetics and Molecular Biology(all)

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