Fatty liver in familial hypobetalipoproteinemia: Triglyceride assembly into VLDL particles is affected by the extent of hepatic steatosis

Maurizio Averna, Tariq S. K. Tanoli, Dmitriy A. Yablonskiy, Nizar Elias, Maurizio Averna, Bruce W. Patterson, Pin Yue, Gustav Schonfeld, Joseph Ackerman

Risultato della ricerca: Article

104 Citazioni (Scopus)

Abstract

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. Nonplasma 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.
Lingua originaleEnglish
pagine (da-a)470-478
Numero di pagine9
RivistaJournal of Lipid Research
Volume44
Stato di pubblicazionePublished - 2003

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Hypobetalipoproteinemias
Fatty Liver
Liver
Triglycerides
Fats
Palmitates
Apolipoproteins B
Obesity
Insulin
Familial Hypobetalipoproteinemia
Magnetic resonance spectroscopy
Plasmas
Viscera
Waist Circumference
Glucose Tolerance Test
Area Under Curve
Mass Spectrometry
Body Mass Index
Mass spectrometry
Magnetic Resonance Spectroscopy

All Science Journal Classification (ASJC) codes

  • Biochemistry
  • Cell Biology
  • Endocrinology

Cita questo

Averna, M., Tanoli, T. S. K., Yablonskiy, D. A., Elias, N., Averna, M., Patterson, B. W., ... Ackerman, J. (2003). Fatty liver in familial hypobetalipoproteinemia: Triglyceride assembly into VLDL particles is affected by the extent of hepatic steatosis. Journal of Lipid Research, 44, 470-478.

Fatty liver in familial hypobetalipoproteinemia: Triglyceride assembly into VLDL particles is affected by the extent of hepatic steatosis. / Averna, Maurizio; Tanoli, Tariq S. K.; Yablonskiy, Dmitriy A.; Elias, Nizar; Averna, Maurizio; Patterson, Bruce W.; Yue, Pin; Schonfeld, Gustav; Ackerman, Joseph.

In: Journal of Lipid Research, Vol. 44, 2003, pag. 470-478.

Risultato della ricerca: Article

Averna, M, Tanoli, TSK, Yablonskiy, DA, Elias, N, Averna, M, Patterson, BW, Yue, P, Schonfeld, G & Ackerman, J 2003, 'Fatty liver in familial hypobetalipoproteinemia: Triglyceride assembly into VLDL particles is affected by the extent of hepatic steatosis', Journal of Lipid Research, vol. 44, pagg. 470-478.
Averna, Maurizio ; Tanoli, Tariq S. K. ; Yablonskiy, Dmitriy A. ; Elias, Nizar ; Averna, Maurizio ; Patterson, Bruce W. ; Yue, Pin ; Schonfeld, Gustav ; Ackerman, Joseph. / Fatty liver in familial hypobetalipoproteinemia: Triglyceride assembly into VLDL particles is affected by the extent of hepatic steatosis. In: Journal of Lipid Research. 2003 ; Vol. 44. pagg. 470-478.
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title = "Fatty liver in familial hypobetalipoproteinemia: Triglyceride assembly into VLDL particles is affected by the extent of hepatic steatosis",
abstract = "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. Nonplasma 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.",
author = "Maurizio Averna and Tanoli, {Tariq S. K.} and Yablonskiy, {Dmitriy A.} and Nizar Elias and Maurizio Averna and Patterson, {Bruce W.} and Pin Yue and Gustav Schonfeld and Joseph Ackerman",
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T1 - Fatty liver in familial hypobetalipoproteinemia: Triglyceride assembly into VLDL particles is affected by the extent of hepatic steatosis

AU - Averna, Maurizio

AU - Tanoli, Tariq S. K.

AU - Yablonskiy, Dmitriy A.

AU - Elias, Nizar

AU - Averna, Maurizio

AU - Patterson, Bruce W.

AU - Yue, Pin

AU - Schonfeld, Gustav

AU - Ackerman, Joseph

PY - 2003

Y1 - 2003

N2 - 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. Nonplasma 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.

AB - 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. Nonplasma 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.

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