6533b7d7fe1ef96bd126907a
RESEARCH PRODUCT
Fatty liver in familial hypobetalipoproteinemia: triglyceride assembly into VLDL particles is affected by the extent of hepatic steatosis
Maurizio AvernaNizar EliasTariq TanoliPin YueDmitriy A. YablonskiyGustav SchonfeldJoseph J. H. AckermanBruce W. Pattersonsubject
MaleVery low-density lipoproteinSettore MED/09 - Medicina InternaApolipoprotein Bmedicine.medical_treatmentPalmitic AcidLipoproteins VLDLSeverity of Illness IndexTriglyceridevery low density lipoprotein assemblyBiochemistryBody Mass IndexMagnetic resonence spectroscopy; Nonalcoholic fatty liver; Nonesterified fatty acids; Very low density lipoprotein assembly; Adult; Body Mass Index; Dietary Fats; Fatty Liver; Female; Glucose Tolerance Test; Humans; Hypobetalipoproteinemias; Insulin Resistance; Lipoproteins VLDL; Liver Diseases; Male; Middle Aged; Obesity; Palmitic Acids; Severity of Illness Index; Triglycerides; EndocrinologyHypobetalipoproteinemiaschemistry.chemical_compoundEndocrinologyDietary FatGlucose tolerance testmedicine.diagnostic_testbiologyChemistryLiver DiseasesLiver DiseaseFatty liverMiddle AgedFemalemagnetic resonence spectroscopyHumanAdultmedicine.medical_specialtyPalmitic Acidsnonesterified fatty acidsQD415-436Internal medicinemedicinenonalcoholic fatty liverHumansObesityTriglyceridesTriglycerideInsulinNonesterified fatty acidCell BiologyGlucose Tolerance Testmedicine.diseaseDietary FatsFatty LiverEndocrinologybiology.proteinHypobetalipoproteinemiaInsulin ResistanceSteatosisHypobetalipoproteinemiadescription
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.
| year | journal | country | edition | language |
|---|---|---|---|---|
| 2003-03-01 | Journal of Lipid Research |