s / Pancreatology 13 (2013) S2–S98 S18 anastomosis and 69 patients had duodenoduodenal anastomosis. Overall patient survival is 85%, 61% of the patients have a good pancreas graft function, 75% present good kidney graft function after 5 years post transplantation. The patients’ demographic data and post-surgical complications were analysed. Conclusion: In our experience duodenoduodenal anastomosis is a valuable type of exocrine drainage that provides some added advantages compared to enteric drainage. It enables easy and noninvasive endoscopic access to the transplanted duodenum and may be helpful in monitoring the graft survival and preventing some of the possible complications. O-49 Abstract id: 168. The CEL-MODY syndrome of diabetes and pancreatic exocrine dysfunction: A frame-shift mutation in the CEL gene causes cellular reuptake of secreted CEL-MUT protein Bente Berg Johansson , Janniche Torsvik , Michael Marie , Karianne Fjeld , Stefan Johansson , Jaakko Saraste , P al Njolstad , Anders Molven . 1 KG Jebsen Center for Diabetes Research, Department of Clinical Medicine, University of Bergen, Bergen, Norway Department of Biomedicine, University of Bergen, Bergen, Norway KG Jebsen Center for Diabetes Research, Department of Clinical Medicine, University of Bergen, Bergen, Norway and Center for, Norway KG Jebsen Center for Diabetes Research, Department of Clinical Medicine, University of Bergen, Bergen, Norway Department of Biomedicine, University of Bergen, Bergen, Norway Introduction: CEL-MODY is a disease characterized by diabetes, pancreatic lipomatosis and exocrine dysfunction. It is caused by dominant frame-shift mutations (c.1686delT/, p.Val563CysfsX111) in the carboxylester lipase gene (CEL), which is highly expressed in pancreatic acinar cells. In a previous study, we have proposed that CEL-MODY is a protein misfolding disease involving a negative gain-of-function effect of the mutant protein. Aims: We aimed to study the subcellular distribution of the CEL proteins. Materials & methods: Stably transfected HEK293 cells expressing wild-type (WT) and mutant (MUT) CEL were used as a model system to investigate secretion, degradation and intracellular localization of the proteins by microscopy and biochemical methods Results: In the present study we have investigated the intracellular distribution of the mutant (CEL-MUT) and wild-type (CEL-WT) CEL proteins in cell line models. By fluorescent immunostaining and confocal microscopy CEL-WT was found to localise typically for secreted proteins in the ER and Golgi, whereas electronmicroscopy demonstrated the presence of large CEL-MUT aggregates at the plasma membrane and in the lumen of single-membrane vacuoles in the cytoplasm. Experiments in cells devoid of CEL expression indicated that the CEL-MUT protein presented extracellularly could be internalized via endocytosis and degraded in a lysosome-associated (Lamp1-positive) organelle. Conclusion: Our results suggest that the aggregated forms of extracellular CEL-MUT can be cleared by cell-mediated uptake and degradation. This might represent amechanism of preventing cells from the exposure to potentially toxic CEL aggregates that eventually causes exocrine deficiency and diabetes. O-50 Abstract id: 229. Deoxysphingolipids, a novel biomarker for type 2 diabetes, are cytotoxic for insulin-producing cells Sabrina Sonda , Richard A. Z€ ullig , Thorsten Hornemann , Alaa Othman , Tanja G€ untert , Omolara O. Ogunshola , Rolf Graf . 1 Swiss HPB Center, Visceral & Transplantation Surgery, University Hospital Zurich, Switzerland Division of Endocrinology, Diabetes and Clinical Nutrition, University Hospital, Zurich, Switzerland 3 Institute for Clinical Chemistry, University Hospital Zurich, Switzerland 4 Institute of Veterinary Physiology, University of Zurich, Switzerland Introduction: The prevalence of diabetes mellitus has been rising dramatically worldwide as a consequence of the epidemic spreading of type 2 diabetic patients. Recently, elevated plasma levels of deoxysphingoid bases, including deoxysphinganine (doxSA), have been identified as a novel biomarker for the disease. These atypical sphingolipids lack the C1-hydroxyl group and thus cannot be efficiently catabolized via the canonical degradation pathway. Aims: In this study, we analyzed whether deoxysphingolipids directly compromise the functionality of pancreatic b-cell. Patients &methods: The effect of deoxysphingolipids was analyzed in insulin producing Ins-1 cells and primary islets isolated from Wistar rats. Cellular viability was analyzed by biochemical and RT-PCR array approaches. Intracellular lipid composition was quantified by mass spectrometry. Cytoskeleton dynamics and phosphorylation of signaling molecules were evaluated by immunostaining and western blotting. b-cell functionality was assessed by quantifying glucose-stimulated insulin secretion. Results: Treatment with doxSA reduced cellular metabolic activity and insulin secretion in a dose dependent and irreversible manner. DoxSAinduced cytotoxicity had both necrotic and apoptotic characteristics and was accompanied by disassembly of actin cytoskeleton, without alterations in the microtubule pattern. DoxSA incubation increased the cellular levels of deoxyceramides and inhibition of ceramide synthase improved cellular viability. Analyses of signaling pathways identified JNK and p38 MAPK as mediators of cytotoxicity. Conclusion: Our results revealed that doxSA is a cytotoxic lipid for insulin-producing cells, suggesting that increased levels of this sphingolipid observed in diabetic patients likely contribute to the reduced functionality of b-cells. Thus, targeting deoxysphingolipid synthesis may implement the currently available therapies of diabetes. O-51 Abstract id: 250. Autoimmune pancreatitis in Sweden Stephan L. Haas , Vivi Liu , Aleksandra Hedstr€ om , Nils Albiin , Nikolaos Kartalis , Marco Del Chiaro , Ralf Segersv€ ard , Caroline Verbeke , J.-