PMID- 22288597 OWN - NLM STAT- MEDLINE DCOM- 20120322 LR - 20221207 IS - 1365-2249 (Electronic) IS - 0009-9104 (Print) IS - 0009-9104 (Linking) VI - 167 IP - 3 DP - 2012 Mar TI - Induction of elastin expression in vascular endothelial cells relates to hepatoportal sclerosis in idiopathic portal hypertension: possible link to serum anti-endothelial cell antibodies. PG - 532-42 LID - 10.1111/j.1365-2249.2011.04530.x [doi] AB - Hepatoportal sclerosis accompanied by dense elastic fibre deposition is generally regarded as the primary lesion in the development of idiopathic portal hypertension (IPH). This study was performed to clarify the mechanism of elastic fibre deposition in the peripheral portal tracts of IPH liver in relation to serum anti-endothelial cell antibodies (AECA). In-vitro experiments were performed using human dermal microvascular endothelial cells (HMVEC) and patients' sera. The presence of serum AECA was assayed by a cell-based enzyme-linked immunosorbent assay (ELISA) using HMVEC. Immunohistochemical analysis of elastin was performed using liver tissue sections of IPH patients. IPH sera contained one or more AECA that could bind to the vascular endothelial cells of the peripheral portal tracts of the liver. When the value of AECA greater than the mean +/- 2 standard deviations of healthy controls was regarded as positive, the positive detection rate of either immunoglobulin (Ig)G, IgA or IgM AECA in IPH sera was 30% (10 of 33 cases). IPH sera induced the expression of elastin in HMVEC, which appeared to be associated with the presence of AECA. Apoptosis was also induced in HMVEC by the stimulation with IPH sera. In vivo, elastin expression was observed in the endothelial cells of the peripheral portal tracts of IPH livers in a proportion of cases. The disease pathogenesis of IPH seems to be heterogeneous, and this study elucidated a possible contribution of the induction of elastin expression in the portal vessels to hepatoportal sclerosis of IPH, which might be linked to serum AECA as a causative factor. CI - (c) 2011 The Authors. Clinical and Experimental Immunology (c) 2011 British Society for Immunology. FAU - Sato, Y AU - Sato Y AD - Department of Human Pathology, Kanazawa University Graduate School of Medicine, Kanazawa, Japan. FAU - Ren, X S AU - Ren XS FAU - Harada, K AU - Harada K FAU - Sasaki, M AU - Sasaki M FAU - Morikawa, H AU - Morikawa H FAU - Shiomi, S AU - Shiomi S FAU - Honda, M AU - Honda M FAU - Kaneko, S AU - Kaneko S FAU - Nakanuma, Y AU - Nakanuma Y LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - England TA - Clin Exp Immunol JT - Clinical and experimental immunology JID - 0057202 RN - 0 (Autoantibodies) RN - 0 (DNA Primers) RN - 0 (RNA, Messenger) RN - 9007-58-3 (Elastin) SB - IM MH - Apoptosis MH - Autoantibodies/*blood MH - Base Sequence MH - Case-Control Studies MH - Cells, Cultured MH - DNA Primers/genetics MH - Elastin/*biosynthesis/genetics MH - Endothelial Cells/*immunology/*metabolism MH - Humans MH - Hypertension, Portal/*etiology/immunology/metabolism/pathology MH - Immunohistochemistry MH - In Vitro Techniques MH - Liver Cirrhosis/*etiology/immunology/metabolism/pathology MH - Pancytopenia/*etiology/immunology/metabolism/pathology MH - Portal Vein/*pathology MH - RNA, Messenger/genetics/metabolism MH - Sclerosis MH - Splenomegaly/*etiology/immunology/metabolism/pathology MH - Idiopathic Noncirrhotic Portal Hypertension PMC - PMC3374286 EDAT- 2012/02/01 06:00 MHDA- 2012/03/23 06:00 PMCR- 2013/03/01 CRDT- 2012/02/01 06:00 PHST- 2012/02/01 06:00 [entrez] PHST- 2012/02/01 06:00 [pubmed] PHST- 2012/03/23 06:00 [medline] PHST- 2013/03/01 00:00 [pmc-release] AID - 10.1111/j.1365-2249.2011.04530.x [doi] PST - ppublish SO - Clin Exp Immunol. 2012 Mar;167(3):532-42. doi: 10.1111/j.1365-2249.2011.04530.x.