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Details on Person The microsomal triglyceride transfer protein (MTTP) is requi...

Class:IdSummation:9737768
_displayNameThe microsomal triglyceride transfer protein (MTTP) is requi...
_timestamp2021-07-22 15:11:04
created[InstanceEdit:9737790] Jassal, Bijay, 2021-07-22
literatureReference[LiteratureReference:9737779] A 30-amino acid truncation of the microsomal triglyceride transfer protein large subunit disrupts its interaction with protein disulfide-isomerase and causes abetalipoproteinemia
[LiteratureReference:9737767] An inhibitor of the microsomal triglyceride transfer protein inhibits apoB secretion from HepG2 cells
[LiteratureReference:9737766] Microsomal triglyceride transfer protein (MTP) inhibitors: discovery of clinically active inhibitors using high-throughput screening and parallel synthesis paradigms
[LiteratureReference:9737763] Inhibition of microsomal triglyceride transfer protein alone or with ezetimibe in patients with moderate hypercholesterolemia
[LiteratureReference:9737776] Efficacy and safety of a microsomal triglyceride transfer protein inhibitor in patients with homozygous familial hypercholesterolaemia: a single-arm, open-label, phase 3 study
[LiteratureReference:9737787] Correction to: Lomitapide - a Microsomal Triglyceride Transfer Protein Inhibitor for Homozygous Familial Hypercholesterolemia
modified[InstanceEdit:9737801] Jassal, Bijay, 2021-07-22
[InstanceEdit:9737803] Jassal, Bijay, 2021-07-22
textThe 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).
(summation)[Reaction:9737780] MTTP binds lomitapide [Homo sapiens]
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