Reactome | LMF1,2 bind LIPC dimer

LMF1,2 bind LIPC dimer

Stable Identifier

R-HSA-6785181

Type

Reaction [binding]

Species

Homo sapiens

Compartment

endoplasmic reticulum lumen, endoplasmic reticulum membrane

ReviewStatus

5/5

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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 (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
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