Lipase maturation factor 1: structure and role in lipase folding and assembly.

ABSTRACT:

Purpose of review

Lipase maturation factor 1 (LMF1) is a membrane-bound protein located in the endoplasmic reticulum. It is essential to the folding and assembly (i.e., maturation) of a selected group of lipases that include lipoprotein lipase, hepatic lipase and endothelial lipase. The purpose of this review is to examine recent studies that have begun to elucidate the structure and function of LMF1 and to place it in the context of lipase folding and assembly.

Recent findings

Recent studies identified mutations in LMF1 that cause combined lipase deficiency and hypertriglyceridemia in humans. These mutations result in the truncation of a large, evolutionarily conserved domain (DUF1222), which is essential for interaction with lipases and their attainment of enzymatic activi

SUBMITTER: Doolittle MH 

PROVIDER: S-EPMC3924775 | biostudies-literature | 2010 Jun

REPOSITORIES: biostudies-literature