Microsomal triglyceride transfer protein large subunit

UniProt P55157 Organism Homo sapiens Gene MTP, MTTP

Also known as: MTP, MTTP

Function

Catalyzes the transport of triglyceride, cholesteryl ester, and phospholipid between phospholipid surfaces. Required for the assembly and secretion of plasma lipoproteins that contain apolipoprotein B. May be involved in regulating cholesteryl ester biosynthesis in cells that produce lipoproteins (By similarity).

Classification

Family (Pfam): PF19444 MTP_lip_bd, PF01347 Vitellogenin_N
InterPro: Lipid_transp_b-sht_shell, Lipovitellin_superhlx_dom, MTP_lip-bd, MTTP, Vitellinogen_b-sht_N, Vitellogenin_N
Functional cluster: Ribosomal Proteins & RNA-Processing Enzymes

Experimental structures · PDB · 2

6I7S X-ray 2.50A
8EOJ EM 3.07A

A predicted model is available from AlphaFold.

Gene Ontology · 31

Disease associations

abetalipoproteinemia MONDO:0008692

Drugs targeting this protein · 3

LOMITAPIDE MESYLATE inhibitor
IMPLITAPIDE inhibitor
SLX-4090 inhibitor

Related proteins · sequence + function similarity

Co-cited proteins · studied together in the literature

Microsomal triglyceride transfer protein large subunit 3 shared papers
Apolipoprotein B-100 3 shared papers
Microsomal triglyceride transfer protein large subunit 2 shared papers
Protein disulfide-isomerase 3 shared papers
Microsomal triglyceride transfer protein large subunit 2 shared papers
Microsomal triacylglycerol transfer protein 1 shared papers

Literature · 24 cited papers

Novel APOB missense variants, A224T and V925L, in a black South African woman with marked hypocholesterolemia. J. Clin. Lipidol. · 2016
A novel abetalipoproteinemia missense mutation highlights the importance of N-Terminal beta-barrel in microsomal triglyceride transfer protein function. Circ. Cardiovasc. Genet. · 2015
Novel missense MTTP gene mutations causing abetalipoproteinemia. Biochim. Biophys. Acta · 2014
An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome. J. Proteomics · 2014
Loss of both phospholipid and triglyceride transfer activities of microsomal triglyceride transfer protein in abetalipoproteinemia. J. Lipid Res. · 2013
Molecular and functional analysis of two new MTTP gene mutations in an atypical case of abetalipoproteinemia. J. Lipid Res. · 2012
Initial characterization of the human central proteome. BMC Syst. Biol. · 2011
Phospholipid transfer activity of microsomal triacylglycerol transfer protein is sufficient for the assembly and secretion of apolipoprotein B lipoproteins. J. Biol. Chem. · 2006
Transfer of cholesteryl esters and phospholipids as well as net deposition by microsomal triglyceride transfer protein. J. Lipid Res. · 2005
Generation and annotation of the DNA sequences of human chromosomes 2 and 4. Nature · 2005
The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res. · 2004
Hypobetalipoproteinemia with an apparently recessive inheritance due to a 'de novo' mutation of apolipoprotein B. Biochim. Biophys. Acta · 2004
… and 12 more in the literature graph

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