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.
- 2D DIGE
- Cystathionine β-synthase
- Transcriptional regulation
ASJC Scopus subject areas
- Biochemistry, Genetics and Molecular Biology(all)