Lipoprotein lipase deficiency with pancreatitis in mink: biochemical characterization and pathology
| Autor: | G Olivecrona, Y Shen, Bjørn O. Christophersen, K Nordstoga, T Olivecrona |
|---|---|
| Rok vydání: | 1997 |
| Předmět: |
medicine.medical_specialty
Lipoprotein lipase Pathology biology Chemistry animal diseases Hypertriglyceridemia QD415-436 Cell Biology medicine.disease Biochemistry Lipoprotein lipase deficiency Endocrinology Internal medicine biology.animal medicine biology.protein Pancreatitis lipids (amino acids peptides and proteins) Hepatic lipase Lipase Mink Lipoprotein |
| Zdroj: | Journal of Lipid Research, Vol 38, Iss 5, Pp 837-846 (1997) |
| ISSN: | 0022-2275 |
| DOI: | 10.1016/s0022-2275(20)37209-6 |
| Popis: | A severe hyperlipemia in mink, with a pattern that suggested recessive inheritance, was observed at a farm in Norway. On a normal mink diet, affected animals had grossly elevated levels of plasma triglycerides which decreased towards normal on a low-fat diet. Normal minks had the main part of their plasma cholesterol in the HDL fraction. Affected minks, although severely hypertriglyceridaemic, had almost normal levels of both LDL and HDL. Affected minks frequently had lipogranulomas in the mesentery and the pancreas. The lipogranulomatous tissue contained spaces filled with an amorphous, sudanophilic substance with many foamy macrophages in the fibrous tissue between the lesions. Separation of postheparin plasma on heparin-agarose revealed that the affected minks had no detectable lipoprotein lipase activity but normal activity of hepatic lipase. Both normal and affected minks had inactive lipoprotein lipase protein in pre- and post-heparin plasma. This protein, which eluted before the active lipase from heparin-agarose, probably corresponds to lipase monomers. The presence of lipoprotein lipase mass in the affected minks, but no activity, indicates that there might be a point mutation in the lipase gene. The minks provide a new animal model for studies on pancreatitis induced by hypertriglyceridemia and on lipoprotein metabolism in the lipoprotein lipase-deficient state and show features similar to those found in human hyperlipoproteinemia type I. |
| Databáze: | OpenAIRE |
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