title: Comparative proteomics study on liver mitochondria of primary biliary cirrhosis mouse model.
abstract: BACKGROUND
  Primary biliary cirrhosis (PBC) is a liver specific chronic disease with unclear pathogenesis, especially for the early stage molecular events. The mitochondrion is a multi-functional organelle associated with various diseases including PBC. The purpose of this study was to discover the alterations in the mitochondria proteome using an early stage PBC mouse model for revealing the possible pathogenesis mechanisms in the early stages of PBC.
  
  
  METHODS
  Mouse model of early stage of PBC was constructed by consecutive administration of poly I:C. Mitochondria of mouse models and controls were purified and comparative proteomics was performed by iTRAQ technology. Then, differentially expressed proteins were validated by western blotting.
  
  
  RESULTS
  In total 354 proteins that satisfied the criteria for comparative proteomics study were identified. Of them, nine proteins were downregulated and 20 were up-regulated in liver mitochondria of PBC mouse model. Most differentially expressed proteins are associated with oxidation-reduction and lipid metabolism, and some are involved in the biosynthesis of steroid hormone and primary bile acid. Interestingly, four proteins (HCDH, CPT I, DECR, ECHDC2) involved in the fatty acid beta-oxidation were all upregulated.
  
  
  CONCLUSIONS
  iTRAQ is a powerful tool for comparative proteomics study of PBC mouse model and differentially expressed proteins in mitochondria proteome of PBC mouse model provide insights for the pathogenesis mechanism at early stage of PBC.
authors.affiliation: Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing 100032, PR China.
authors.forename: Guang
authors.initials: G
authors.lastname: Song
authors.forename: Chaojun
authors.initials: C
authors.lastname: Hu
authors.forename: Huishan
authors.initials: H
authors.lastname: Zhu
authors.forename: Xi
authors.initials: X
authors.lastname: Li
authors.forename: Liying
authors.initials: L
authors.lastname: Zhao
authors.forename: Renfang
authors.initials: R
authors.lastname: Zhou
authors.forename: Xuan
authors.initials: X
authors.lastname: Zhang
authors.forename: Fengchun
authors.initials: F
authors.lastname: Zhang
authors.forename: Lin
authors.initials: L
authors.lastname: Wu
authors.forename: Yongzhe
authors.initials: Y
authors.lastname: Li
mesh_terms: D000818:Animals; D015153:Blotting, Western; D004195:Disease Models, Animal; D015536:Down-Regulation; D050356:Lipid Metabolism; D008099:Liver / Q000378:metabolism*; D008105:Liver Cirrhosis, Biliary / Q000139:chemically induced / Q000378:metabolism*; D051379:Mice; D008810:Mice, Inbred C57BL; D008928:Mitochondria / Q000378:metabolism*; D010084:Oxidation-Reduction; D011070:Poly I-C; D040901:Proteomics; D019032:Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization; D015854:Up-Regulation
chemical_list: D011070:Poly I-C