@article{c8e6e827631a45739e0e57de767d7f7d,
title = "2D DIGE proteomic analysis highlights delayed postnatal repression of α-fetoprotein expression in homocystinuria model mice",
abstract = "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 of Cbs-/- 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-/- mice. However, the repression was delayed in Cbs-/- mice, causing high postnatal AFP levels, which may relate to transcriptional repression of most plasma proteins originating from liver and the observed hepatic dysfunction.",
keywords = "2D DIGE, Cystathionine β-synthase, MALDI-TOF/MS, Proteomics, Transcriptional regulation, α-Fetoprotein",
author = "Shotaro Kamata and Noriyuki Akahoshi and Isao Ishii",
note = "Funding Information: This work was supported by the Grants-in-Aid for Scientific Research ( 25460072 and 25220103 ) and the Program for Strategic Research Foundation at Private Universities (2011–2015) from the Ministry of Education, Culture, Sports, Science, and Technology (MEXT) of Japan; and Program for the Advancement of Next Generation Research and Keio Gijuku Fukuzawa Memorial Fund for the Advancement of Education and Research from the Keio University (to I.I.). S.K. is a research fellow of Japan Society for the Promotion of Science. Publisher Copyright: {\textcopyright} 2015 The Authors.",
year = "2015",
doi = "10.1016/j.fob.2015.06.008",
language = "English",
volume = "5",
pages = "535--541",
journal = "FEBS Open Bio",
issn = "2211-5463",
publisher = "Elsevier BV",
}