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Lipase maturation factor 1 (LMF1) is a membrane-bound protein located in the endoplasmic reticulum, essential for the folding and assembly of a selected group of secreted, dimeric lipases, including lipoprotein lipase, hepatic lipase, and endothelial lipase[1][2][3]. LMF1 interacts with enzymes involved in N-linked glycosylation and endoplasmic reticulum-resident oxidoreductases, supporting the formation and maintenance of correct disulfide bonds during protein maturation. LMF1 also assists the secretion of other disulfide-rich oligomeric proteins such as fibronectin and LDL receptor[2][3]. Mutations in LMF1 can cause combined lipase deficiency and severe hypertriglyceridemia in humans due to impaired lipase activity, confirming its crucial role in human lipid metabolism and redox homeostasis in the ER[1][2][3].
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