MTTP binds lomitapide

Stable Identifier
R-HSA-9737780
Type
Reaction [binding]
Species
Homo sapiens
Compartment
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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

Cuchel, M, Bloedon, LT, Szapary, PO, Kolansky, DM, Wolfe, ML, Sarkis, A, Millar, JS, Ikewaki, K, Siegelman, ES, Gregg, RE, Rader, DJ

N Engl J Med 2007
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