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
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