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. 2011 Feb;163(2):147-56.
doi: 10.1111/j.1365-2249.2010.04288.x. Epub 2010 Nov 22.

Autoantibodies to GW bodies and other autoantigens in primary biliary cirrhosis

L M Stinton  1 , M Swain, R P Myers, A A Shaheen, M J Fritzler
Affiliations

Autoantibodies to GW bodies and other autoantigens in primary biliary cirrhosis

L M Stinton et al. Clin Exp Immunol. 2011 Feb.

Abstract

Autoantibodies to intracellular targets in mitochondria and nuclei are serological hallmarks of primary biliary cirrhosis (PBC). One of the most recently identified cellular targets of PBC autoantibodies is a novel cytoplasmic structure referred to as GW bodies [GWB, G (glycine) W (tryptophan)-containing bodies (GWB)]. GWB are indentified as discrete cytoplasmic domains that are involved in mRNA processing via the RNA interference (RNAi) pathway. Key components of GWB include the proteins GW182, Ago2, RNA-associated protein 55 (RAP55) and Ge-1/Hedls. The primary objective was to study the frequency and clinical association of antibodies directed to GWB components, in 109 PBC patients. Autoantibodies to mitochondrial antigen-pyruvate dehydrogenase complex (M2), branched-chain 2-oxo-acid dehydrogenase complex and 2-oxo glutarate dehydrogenase complex (3E-BPO), gp210, sp100, promyelocytic leukaemia cell antigen (PML) and liver kidney microsomal-1 antigen (LKM-1) were detected by a line immunoassay and antibodies to GWB (GW182, RAP55, Ge-1, GW2, GW3) and glutamate receptor interacting protein (GRIP)-associated protein-1 (GRASP-1), by an addressable laser bead immunoassay (ALBIA). The most common GWB autoantigen targets were: RAP55-28%, GW182-12%, GW2-2% and antibodies to GRASP-1-17%. By comparison, the frequency of reactivity to established PBC autoantigens was: gp210, 27%; sp100, 27% and PML, 17%. None of the autoantibodies were associated with differences in Mayo risk score or liver decompensation. This study is the first study to show that antibodies to RAP55, GW182 and GRASP-1 are the most common GWB targets in PBC.

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Figures

Fig. 1
Fig. 1
Indirect immunofluorescence (IIF) of a human primary biliary cirrhosis (PBC) serum on a conventional clinical laboratory screening substrate, human epidermoid cancer cells (HEp-2), displayed a cytoplasmic staining pattern typical of anti-mitochondrial antibodies (a). Co-localization with a monoclonal antibody 4B6 (anti-GW182) (b) revealed that the some sera also had a cytoplasmic dot staining (CDS) pattern that was masked by the anti-mitochondrial antibodies (AMA) staining but that co-localized (arrows) with GWB, G (glycine) W (tryptophan)-containing bodies (GWBs) (c). Reactivity with GWB was substantiated by addressable laser bead immunoassay (ALBIA) when it was found that these sera had high titres of antibodies to components of GWB (i.e. RAP55, GW182).
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