| Summary: | 碩士 === 長庚大學 === 臨床醫學研究所 === 95 === 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 six 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 two subjects without liver disorder were used as normal reference for two-dimensional gel electrophoresis and for identification of protein spots by mass spectrometric analysis. Plasma samples from another three normal subjects (with a total of five) were used for nephelometric quantification of immunoglobulin kappa light chain in comparison with patients’ samples.
Results: Among the protein spots detected, ranging from 6–200 kDa mass with pIs of 3–10, significant up-regulation of immunoglobulin kappa light chain was found at late stage of BA, which was subsequently confirmed by nephelometric analysis. Conversely, significant decrease of apolipoprotein (Apo) A-I and C-II, haptoglobin α2 and β 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.
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