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.

MICROSOMAL TRIGLYCERIDE TRANSFER PROTEIN

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).

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

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

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.