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Journal of Lipid Research, Vol. 45, 941-947, May 2004
Copyright © 2004 by American Society for Biochemistry and Molecular Biology

Fatty liver in familial hypobetalipoproteinemia

Tariq Tanoli, Pin Yue, Dmitriy Yablonskiy and Gustav Schonfeld¹

Departments of Internal Medicine and Radiology, Washington University School of Medicine, St. Louis, MO

¹ To whom correspondence should be addressed. e-mail: gschonfe{at}wustl.edu

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.

Supplementary key words abdominal adipose tissue • insulin resistance • apolipoprotein B

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