title: Characterization of plasma proteome in biliary atresia.
abstract: BACKGROUND
  Biliary atresia (BA) is a disorder during infancy with unknown etiology in which progression frequently leads to liver cirrhosis. Plasma proteome is characterized in this study.
  
  
  METHODS
  Twelve paired plasma samples from 6 children with BA who received surgical correction at early stage and then liver transplantation at late stage of liver cirrhosis were studied. Plasma samples from 2 subjects without liver disorder were used as normal reference for 2-dimensional gel electrophoresis and for identification of protein spots by mass spectrometric analysis. Plasma samples from another 3 normal subjects (with a total of 5) were used for nephelometric quantification of immunoglobulin kappa light chain in comparison with patients' samples.
  
  
  RESULTS
  Among the protein spots detected, ranging from 6 to 200 kDa mass with pIs of 3-10, significant up-regulation of immunoglobulin kappa light chain was found at the late stage of BA, which was subsequently confirmed by nephelometric analysis. Conversely, significant decrease of apolipoprotein (Apo) A-I and C-II, haptoglobin alpha2 and beta chain, and transthyretin were detected during the progression of BA.
  
  
  CONCLUSIONS
  Increased immunoglobulin kappa light chain detected in late-stage BA characterizes adverse immune modulation in this disorder. Decreased apolipoproteins, haptoglobin and transthyretin levels might be potential markers of progressive liver injury, fibrosis and defective lipid metabolism in BA.
authors.affiliation: Graduate Institute of Clinical Medical Sciences, Chang Gung University, Taiwan.
authors.forename: Chi Wei
authors.initials: CW
authors.lastname: Lee
authors.forename: Meng Yi
authors.initials: MY
authors.lastname: Lin
authors.forename: Wen-Chien
authors.initials: WC
authors.lastname: Lee
authors.forename: Ming Huei
authors.initials: MH
authors.lastname: Chou
authors.forename: Chih Sung
authors.initials: CS
authors.lastname: Hsieh
authors.forename: Shin Ye
authors.initials: SY
authors.lastname: Lee
authors.forename: Jiin Haur
authors.initials: JH
authors.lastname: Chuang
mesh_terms: D001656:Biliary Atresia / Q000097:blood*; D001797:Blood Protein Electrophoresis; D001798:Blood Proteins / Q000378:metabolism*; D002648:Child; D030562:Databases, Protein; D015180:Electrophoresis, Gel, Two-Dimensional; D006801:Humans; D007145:Immunoglobulin kappa-Chains / Q000096:biosynthesis / Q000097:blood; D008103:Liver Cirrhosis / Q000097:blood*; D016031:Liver Transplantation; D020543:Proteome; D015854:Up-Regulation
chemical_list: D001798:Blood Proteins; D007145:Immunoglobulin kappa-Chains; D020543:Proteome