Fatty liver in familial hypobetalipoproteinemia

Fatty liver is frequent in the apolipoprotein B (apoB)-defective genetic form of familial hypobetalipoproteinemia (FHBL), but interindividual variability in liver fat is large. To explain this, we assessed the roles of metabolic factors in 32 affected family members with apoB-defective FHBL and 33 r...

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Main Authors: Tariq Tanoli, Pin Yue, Dmitriy Yablonskiy, Gustav Schonfeld
Format: Article
Language:English
Published: Elsevier 2004-05-01
Series:Journal of Lipid Research
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S0022227520318356
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author Tariq Tanoli
Pin Yue
Dmitriy Yablonskiy
Gustav Schonfeld
author_facet Tariq Tanoli
Pin Yue
Dmitriy Yablonskiy
Gustav Schonfeld
author_sort Tariq Tanoli
collection DOAJ
description Fatty liver is frequent in the apolipoprotein B (apoB)-defective genetic form of familial hypobetalipoproteinemia (FHBL), but interindividual variability in liver fat is large. To explain this, we assessed the roles of metabolic factors in 32 affected family members with apoB-defective FHBL and 33 related and unrelated normolipidemic controls matched for age, sex, and indices of adiposity. Two hour, 75 g oral glucose tests, with measurements of plasma glucose and insulin levels, body mass index, and waist-hip ratios were obtained. Abdominal subcutaneous, intraperitoneal (IPAT), and retroperitoneal adipose tissue masses were quantified by MR imaging, and hepatic fat was quantified by MR spectroscopy. Mean ± SD liver fat percentage values of FHBL and controls were 14.8 ± 12.0 and 5.2 ± 5.9, respectively (P = 0.001). Means for these measures of obesity and insulin action were similar in the two groups. Important determinants of liver fat percentage were FHBL-affected status, IPAT, and area under the curve (AUC) insulin in both groups, but the strongest predictors were IPAT in FHBL (partial R2 = 0.55, P < 0.0002) and AUC insulin in controls (partial R2 = 0.59, P = 0.0001). Regression of liver fat percentage on IPAT fat was significantly greater for FHBL than for controls (P < 0.001).In summary, because apoB-defective FHBL imparts heightened susceptibility to liver triglyceride accumulation, increasing IPAT and insulin resistance exert greater liver fat-increasing effects in FHBL.
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spelling doaj.art-a93f6fb7a65a460daa1e731aeda8a2932022-12-21T22:09:43ZengElsevierJournal of Lipid Research0022-22752004-05-01455941947Fatty liver in familial hypobetalipoproteinemiaTariq Tanoli0Pin Yue1Dmitriy Yablonskiy2Gustav Schonfeld3Departments of Internal Medicine and Radiology, Washington University School of Medicine, St. Louis, MODepartments of Internal Medicine and Radiology, Washington University School of Medicine, St. Louis, MODepartments of Internal Medicine and Radiology, Washington University School of Medicine, St. Louis, MODepartments of Internal Medicine and Radiology, Washington University School of Medicine, St. Louis, MOFatty liver is frequent in the apolipoprotein B (apoB)-defective genetic form of familial hypobetalipoproteinemia (FHBL), but interindividual variability in liver fat is large. To explain this, we assessed the roles of metabolic factors in 32 affected family members with apoB-defective FHBL and 33 related and unrelated normolipidemic controls matched for age, sex, and indices of adiposity. Two hour, 75 g oral glucose tests, with measurements of plasma glucose and insulin levels, body mass index, and waist-hip ratios were obtained. Abdominal subcutaneous, intraperitoneal (IPAT), and retroperitoneal adipose tissue masses were quantified by MR imaging, and hepatic fat was quantified by MR spectroscopy. Mean ± SD liver fat percentage values of FHBL and controls were 14.8 ± 12.0 and 5.2 ± 5.9, respectively (P = 0.001). Means for these measures of obesity and insulin action were similar in the two groups. Important determinants of liver fat percentage were FHBL-affected status, IPAT, and area under the curve (AUC) insulin in both groups, but the strongest predictors were IPAT in FHBL (partial R2 = 0.55, P < 0.0002) and AUC insulin in controls (partial R2 = 0.59, P = 0.0001). Regression of liver fat percentage on IPAT fat was significantly greater for FHBL than for controls (P < 0.001).In summary, because apoB-defective FHBL imparts heightened susceptibility to liver triglyceride accumulation, increasing IPAT and insulin resistance exert greater liver fat-increasing effects in FHBL.http://www.sciencedirect.com/science/article/pii/S0022227520318356abdominal adipose tissueinsulin resistanceapolipoprotein B
spellingShingle Tariq Tanoli
Pin Yue
Dmitriy Yablonskiy
Gustav Schonfeld
Fatty liver in familial hypobetalipoproteinemia
Journal of Lipid Research
abdominal adipose tissue
insulin resistance
apolipoprotein B
title Fatty liver in familial hypobetalipoproteinemia
title_full Fatty liver in familial hypobetalipoproteinemia
title_fullStr Fatty liver in familial hypobetalipoproteinemia
title_full_unstemmed Fatty liver in familial hypobetalipoproteinemia
title_short Fatty liver in familial hypobetalipoproteinemia
title_sort fatty liver in familial hypobetalipoproteinemia
topic abdominal adipose tissue
insulin resistance
apolipoprotein B
url http://www.sciencedirect.com/science/article/pii/S0022227520318356
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AT pinyue fattyliverinfamilialhypobetalipoproteinemia
AT dmitriyyablonskiy fattyliverinfamilialhypobetalipoproteinemia
AT gustavschonfeld fattyliverinfamilialhypobetalipoproteinemia