Fatty liver in familial hypobetalipoproteinemia: triglyceride assembly into VLDL particles is affected by the extent of hepatic steatosis
Autor: | Maurizio Averna, Nizar Elias, Tariq Tanoli, Pin Yue, Dmitriy A. Yablonskiy, Gustav Schonfeld, Joseph J. H. Ackerman, Bruce W. Patterson |
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Přispěvatelé: | Schonfeld, G., Patterson, B., Yablonskiy, D., Tanoli, T., Averna, M., Elias, N., Yue, P., Ackerman, J. |
Jazyk: | angličtina |
Rok vydání: | 2003 |
Předmět: |
Male
Very low-density lipoprotein Settore MED/09 - Medicina Interna Apolipoprotein B medicine.medical_treatment Palmitic Acid Lipoproteins VLDL Severity of Illness Index Triglyceride very low density lipoprotein assembly Biochemistry Body Mass Index Magnetic resonence spectroscopy Nonalcoholic fatty liver Nonesterified fatty acids Very low density lipoprotein assembly Adult Dietary Fats Fatty Liver Female Glucose Tolerance Test Humans Hypobetalipoproteinemias Insulin Resistance Liver Diseases Middle Aged Obesity Palmitic Acids Triglycerides Endocrinology chemistry.chemical_compound Dietary Fat Glucose tolerance test medicine.diagnostic_test biology Chemistry Liver Disease Fatty liver magnetic resonence spectroscopy Human medicine.medical_specialty nonesterified fatty acids QD415-436 Internal medicine medicine nonalcoholic fatty liver Insulin Nonesterified fatty acid Cell Biology medicine.disease biology.protein Hypobetalipoproteinemia Steatosis |
Zdroj: | Journal of Lipid Research, Vol 44, Iss 3, Pp 470-478 (2003) |
ISSN: | 0022-2275 |
Popis: | Familial hypobetalipoproteinemia (FHBL) subjects may develop fatty liver. Liver fat was assessed in 21 FHBL with six different apolipoprotein B (apoB) truncations (apoB-4 to apoB-89) and 14 controls by magnetic resonance spectroscopy (MRS). Liver fat percentages were 16.7 +/- 11.5 and 3.3 +/- 2.9 (mean +/- SD) (P = 0.001). Liver fat percentage was positively correlated with body mass index, waist circumference, and areas under the insulin curves of 2 h glucose tolerance tests, suggesting that obesity may affect the severity of liver fat accumulation in both groups. Despite 5-fold differences in liver fat percentage, mean values for obesity and insulin indexes were similar. Thus, for similar degrees of obesity, FHBL subjects have more hepatic fat. VLDL-triglyceride (TG)-fatty acids arise from plasma and nonplasma sources (liver and splanchnic tissues). To assess the relative contributions of each, [2H2]palmitate was infused over 12 h in 13 FHBL subjects and 11 controls. Isotopic enrichment of plasma free palmitate and VLDL-TG-palmitate was determined by mass spectrometry. Non-plasma sources contributed 51 +/- 15% in FHBL and 37 +/- 13% in controls (P = 0.02). Correlations of liver fat percentage and percent VLDL-TG-palmitate from liver were r = 0.89 (P = 0.0001) for FHBL subjects and r = 0.69 (P = 0.01) for controls. Thus, apoB truncation-producing mutations result in fatty liver and in altered assembly of VLDL-TG. |
Databáze: | OpenAIRE |
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