Lipoprotein subfractions highly associated with renal damage in familial lecithin:cholesterol acyltransferase deficiency
| Autor: | Yasuyuki Aoyagi, Kouju Kamata, Adriaan G. Holleboom, Masayuki Kuroda, Sakiyo Asada, Shizuya Yamashita, Shun Ishibashi, Erik S.G. Stroes, Hideaki Bujo, Yasushi Saito |
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| Přispěvatelé: | Vascular Medicine, Amsterdam Cardiovascular Sciences |
| Jazyk: | angličtina |
| Rok vydání: | 2014 |
| Předmět: |
Adult
Male medicine.medical_specialty food.ingredient Adolescent Lipoproteins Sterol O-acyltransferase Kidney Lecithin Phosphatidylcholine-Sterol O-Acyltransferase chemistry.chemical_compound food Lecithin Cholesterol Acyltransferase Deficiency Internal medicine medicine Humans Enzyme Replacement Therapy Genetic Predisposition to Disease Renal Insufficiency Child Chromatography High Pressure Liquid Aged Lecithin cholesterol acyltransferase deficiency Triglyceride Chemistry Lecithin Acyltransferase Deficiency fungi Middle Aged medicine.disease Recombinant Proteins Proteinuria Endocrinology Phenotype Renal pathology Biochemistry Case-Control Studies Mutation Cholesteryl ester Chromatography Gel Female lipids (amino acids peptides and proteins) Cardiology and Cardiovascular Medicine Biomarkers Lipoprotein |
| Zdroj: | Arteriosclerosis, thrombosis, and vascular biology, 34(8), 1756-1762. Lippincott Williams and Wilkins |
| ISSN: | 1079-5642 |
| DOI: | 10.1161/atvbaha.114.303420 |
| Popis: | Objective— In familial lecithin:cholesterol acyltransferase (LCAT) deficiency (FLD), deposition of abnormal lipoproteins in the renal stroma ultimately leads to renal failure. However, fish-eye disease (FED) does not lead to renal damage although the causative mutations for both FLD and FED lie within the same LCAT gene. This study was performed to identify the lipoproteins important for the development of renal failure in genetically diagnosed FLD in comparison with FED, using high-performance liquid chromatography with a gel filtration column. Approach and Results— Lipoprotein profiles of 9 patients with LCAT deficiency were examined. Four lipoprotein fractions specific to both FLD and FED were identified: (1) large lipoproteins (>80 nm), (2) lipoproteins corresponding to large low-density lipoprotein (LDL), (3) lipoproteins corresponding to small LDL to large high-density lipoprotein, and (4) to small high-density lipoprotein. Contents of cholesteryl ester and triglyceride of the large LDL in FLD (below detection limit and 45.8±3.8%) and FED (20.7±6.4% and 28.0±6.5%) were significantly different, respectively. On in vitro incubation with recombinant LCAT, content of cholesteryl ester in the large LDL in FLD, but not in FED, was significantly increased (to 4.2±1.4%), whereas dysfunctional high-density lipoprotein was diminished in both FLD and FED. Conclusions— Our novel analytic approach using high-performance liquid chromatography with a gel filtration column identified large LDL and high-density lipoprotein with a composition specific to FLD, but not to FED. The abnormal lipoproteins were sensitive to treatment with recombinant LCAT and thus may play a causal role in the renal pathology of FLD. |
| Databáze: | OpenAIRE |
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