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Lipoprotein glomerulopathy

Takao Saito, MD, PhD
Section Editors
Richard J Glassock, MD, MACP
Brad H Rovin, MD
Deputy Editor
Albert Q Lam, MD


Lipoprotein glomerulopathy (LPG) is a unique and rare disorder of renal lipidosis that was first reported in a Japanese patient in 1989 [1]. It is characterized by lipoprotein thrombi in glomeruli, an abnormal plasma lipoprotein profile that resembles type III hyperlipoproteinemia, and a marked increase in serum apolipoprotein E (apo E) concentrations. LPG was subsequently found to be due to mutations of the APOE gene [2]. However, intrinsic renal factors are also involved in the pathogenesis of LPG because of the incomplete penetrance based upon many asymptomatic carriers of APOE variants [3-5].

A review of the clinical features of LPG is presented in this topic review.


Initial reports of familial cases as well as recurrence in transplanted kidneys suggested that a systemically acting genetic factor may underlie LPG. It was also observed that the serum apo E isoform was inconsistent with the APOE genotype among affected individuals.

These findings predicted that an uncharacterized variant(s) of the APOE gene may be responsible for the pathogenesis of this disorder [6]. This was subsequently confirmed as mutations in the APOE gene have been found in all reported cases of LPG in which DNA sequencing has been performed (algorithm 1).

APOE variants — Apolipoprotein E (apo E), an essential apolipoprotein, is a major constituent of various plasma lipoproteins. Plasma lipoprotein levels are determined in part by the binding activities of apo E to the LDL receptor, LDL receptor-related protein, and VLDL receptor. Apo E is composed of 299 amino acids and has a relative molecular mass of 34kDa.

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Literature review current through: Nov 2017. | This topic last updated: Jul 03, 2017.
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