MTTP

MTP encodes the large subunit of the heterodimeric microsomal triglyceride transfer protein. Protein disulfide isomerase (PDI) completes the heterodimeric microsomal triglyceride transfer protein, which has been shown to play a central role in lipoprotein assembly. Mutations in MTP can cause abetalipoproteinemia.

Full Name

MICROSOMAL TRIGLYCERIDE TRANSFER PROTEIN

Function

Catalyzes the transport of triglyceride, cholesteryl ester, and phospholipid between phospholipid surfaces (PubMed:23475612, PubMed:8939939, PubMed:26224785, PubMed:25108285, PubMed:22236406, PubMed:16478722, PubMed:15897609, PubMed:8876250).

Required for the assembly and secretion of plasma lipoproteins that contain apolipoprotein B (PubMed:23475612, PubMed:8939939, PubMed:26224785, PubMed:8876250, PubMed:16478722).

May be involved in regulating cholesteryl ester biosynthesis in cells that produce lipoproteins (By similarity).

Biological Process

Cholesterol homeostasis Source: GO_Central
Chylomicron assembly Source: Reactome
Circadian rhythm Source: Ensembl
Lipid metabolic process Source: ProtInc
Lipoprotein metabolic process Source: GO_Central
Lipoprotein transport Source: Ensembl
Low-density lipoprotein particle remodeling Source: Ensembl
Phospholipid transport Source: UniProtKB
Plasma lipoprotein particle assembly Source: UniProtKB
Protein lipidation Source: Ensembl
Protein secretion Source: UniProtKB
Response to calcium ion Source: Ensembl
Triglyceride metabolic process Source: Ensembl
Triglyceride transport Source: UniProtKB
Very-low-density lipoprotein particle assembly Source: Reactome

Cellular Location

Golgi apparatus
Endoplasmic reticulum
Note: Colocalizes with P4HB/PDI in the endoplasmic reticulum (PubMed:23475612, PubMed:26224785).

Involvement in disease

Abetalipoproteinemia (ABL):
An autosomal recessive disorder of lipoprotein metabolism. Affected individuals produce virtually no circulating apolipoprotein B-containing lipoproteins (chylomicrons, VLDL, LDL, lipoprotein(A)). Malabsorption of the antioxidant vitamin E occurs, leading to spinocerebellar and retinal degeneration.