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Abnormal lipoprotein and apolipoprotein pattern in lipoprotein glomerulopathy
1Second Department of Internal Medicine, Tohoku University School of Medicine, Japan.
Insights
Lipoprotein glomerulopathy involves abnormal lipid buildup in kidney glomeruli. High levels of apolipoprotein E appear linked to this rare renal disease and its characteristic lipoprotein thrombi.
Area of Science:
- Nephrology
- Lipidology
- Pathology
Background:
- Lipoprotein glomerulopathy is a rare renal disease characterized by "lipoprotein thrombi" in glomerular capillaries.
- This condition has been identified in six patients through renal histology.
Purpose of the Study:
- To investigate the clinical and biochemical characteristics of lipoprotein glomerulopathy.
- To explore the association between apolipoprotein E levels, lipoprotein profiles, and the development of lipoprotein thrombi.
Main Methods:
- Analysis of clinical data and renal histology from six patients with lipoprotein glomerulopathy.
- Biochemical assays including lecithin-cholesterol acyltransferase (LCAT) activity, lipoprotein profiling, and plasma apolipoprotein E (apo E) levels.
- Comparison with control groups: hyperlipidemic nephrotic syndrome without thrombi and type IIb hyperlipoproteinemia without renal disease.
Main Results:
- All six patients exhibited proteinuria (1.6-10 g/d) and normal LCAT activity.
- Patients presented with type III hyperlipoproteinemia-like lipoprotein profiles.
- Significantly elevated plasma apo E levels (>10 mg/dL) were observed compared to controls (P < 0.01).
- Apo E isoforms were predominantly rare (five E2/3, one E4/4), distinguishing it from familial type III hyperlipoproteinemia.
Conclusions:
- Elevated apolipoprotein E levels, in conjunction with specific apo E isoforms and lipoprotein metabolic derangements, are strongly associated with lipoprotein glomerulopathy.
- The findings suggest a causal link between apo E-related hyperlipoproteinemia and the formation of lipoprotein thrombi in the renal glomeruli.
- Further research is warranted to elucidate the precise mechanisms connecting apo E metabolism and thrombus formation in this renal disease.
Abstract:
Recently, two cases of renal disease were observed in which there was an abnormal accumulation of lipids, "lipoprotein thrombi," in the glomerular capillary lumen. This disease has been designated as lipoprotein glomerulopathy. Four other cases have been diagnosed independently by renal histology in other clinical laboratories. All six patients showed proteinuria (1.6 to 10 g/d), normal lecithin-cholesterol acyltransferase (LCAT) activity, type III hyperlipoproteinemia-like lipoprotein profiles, and significantly (P less than 0.01) higher levels of plasma apolipoprotein (apo) E (greater than 10 mg/dL) compared with the control patients with hyperlipidemic nephrotic syndrome without lipoprotein thrombi and type IIb hyperlipoproteinemia without renal disease. Lipoprotein glomerulopathy is not familial type III hyperlipoproteinemia (dysbetalipoproteinemia), because apolipoprotein E3 is present. Apo E isoforms were all rare: five cases of E2/3 and one case of E4/4. These results suggest that excessive apo E is associated with apo E isoform and lipoprotein metabolic derangement in such a renal disease. Further studies are needed on the relationship between the apo E hyperlipoproteinemia and the formation of lipoprotein thrombi.
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