LMF1,2 bind LIPC dimer

Stable Identifier
R-HSA-6785181
Type
Reaction [binding]
Species
Homo sapiens
Compartment
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Hepatic triacylglycerol lipase (LIPC, and lipoprotein lipase, LPL) are dimeric lipases (Hill et al. 1997) that hydrolyse triglycerides from circulating lipoproteins thereby playing important roles in lipoprotein remodeling and uptake. LIPC dimer (as well as LPL dimer) binds to an ER membrane protein known as lipase maturation factor 1 (LMF1) which can mediate secretion and enzymatic maturation of these lipases (Doolittle et al. 2009, Babilonia-Rosa & Neher 2014). Another family member, LMF2, may function as LMF1 based on similarity. Defects in LMF1 can cause combined lipase deficiency (CLD; MIM:246650), characterised by hypertriglyceridemia caused by ER retention of both LIPC and LPL (Peterfy et al. 2007).

Literature References
PubMed ID Title Journal Year
24909692 Purification, cellular levels, and functional domains of lipase maturation factor 1

Babilonia-Rosa, MA, Neher, SB

Biochem. Biophys. Res. Commun. 2014
19783858 Lipase maturation factor LMF1, membrane topology and interaction with lipase proteins in the endoplasmic reticulum

Doolittle, MH, Neher, SB, Ben-Zeev, O, Ling-Liao, J, Gallagher, CM, Hosseini, M, Yin, F, Wong, H, Walter, P, Péterfy, M

J. Biol. Chem. 2009
17994020 Mutations in LMF1 cause combined lipase deficiency and severe hypertriglyceridemia

Péterfy, M, Ben-Zeev, O, Mao, HZ, Weissglas-Volkov, D, Aouizerat, BE, Pullinger, CR, Frost, PH, Kane, JP, Malloy, MJ, Reue, K, Pajukanta, P, Doolittle, MH

Nat. Genet. 2007
9379936 Hepatic lipase: high-level expression and subunit structure determination

Hill, JS, Davis, RC, Yang, D, Schotz, MC, Wong, H

Meth. Enzymol. 1997
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