Reactome | MTTP binds lomitapide

MTTP binds lomitapide

Stable Identifier

R-HSA-9737780

Type

Reaction [binding]

Species

Homo sapiens

Compartment

endoplasmic reticulum lumen

ReviewStatus

5/5

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The microsomal triglyceride transfer protein (MTTP) is required for the assembly and secretion of plasma lipoproteins that contain apolipoprotein B. The discovery of a mutation in the MTTP gene causing abetalipoproteinemia (Ricci et al. 1995) was essential for the development of drugs to inhibit MTTP (Jamil et al. 1996, Chang et al. 2002).

The pharmacological inhibition of MTTP by lomitapide is associated with a decrease in LDL cholesterol (LDL-C) and triglycerides (Cuchel et al. 2007). Lomitapide significantly reduces LDL-C in homozygous familial hypercholesterolemia (hFH) (Samaha et al. 2008, Cuchel et al. 2013) when administered concurrently with other lipid-lowering therapies (Stefanutti 2020). Lomitapide can lead to elevated aminotransferase levels and fat accumulation in the liver (Cuchel et al. 2007).

Literature References

PubMed ID	Title	Journal	Year
17215532	Inhibition of microsomal triglyceride transfer protein in familial hypercholesterolemia Sarkis, A, Rader, DJ, Szapary, PO, Bloedon, LT, Gregg, RE, Ikewaki, K, Kolansky, DM, Siegelman, ES, Millar, JS, Wolfe, ML, Cuchel, M	N Engl J Med	2007