Microsomal triglyceride transfer protein (original) (raw)

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Large subunit of microsomal triglyceride transfer protein

MTTP
Available structuresPDBOrtholog search: PDBe RCSB List of PDB id codes6I7S, 8EOJ
Identifiers
Aliases MTTP, ABL, MTP, Microsomal triglyceride transfer protein
External IDs OMIM: 157147; MGI: 106926; HomoloGene: 212; GeneCards: MTTP; OMA:MTTP - orthologs
Gene location (Human)Chromosome 4 (human)Chr.Chromosome 4 (human)[1]Chromosome 4 (human)Genomic location for MTTPGenomic location for MTTPBand4q23Start99,564,081 bp[1]End99,623,997 bp[1]
Gene location (Mouse)Chromosome 3 (mouse)Chr.Chromosome 3 (mouse)[2]Chromosome 3 (mouse)Genomic location for MTTPGenomic location for MTTPBand3 G3|3 64.06 cMStart137,795,615 bp[2]End137,850,729 bp[2]
RNA expression patternBgeeHuman Mouse (ortholog)Top expressed injejunal mucosamucosa of ileumliverduodenumright lobe of livertesticleventricular zonekidney tubulegonadhuman kidneyTop expressed injejunumduodenummigratory enteric neural crest cellleft lobe of liverileumyolk sacspermatocyteintestinal epitheliumcrypt of lieberkuhn of small intestinespermatidMore reference expression dataBioGPSMore reference expression data
Gene ontologyMolecular function protein heterodimerization activity protein binding phospholipid transporter activity lipid binding lipid transporter activity Cellular component receptor complex endoplasmic reticulum lumen endoplasmic reticulum Golgi apparatus basolateral plasma membrane Biological process phospholipid transport lipid transport plasma lipoprotein particle assembly lipid metabolism protein secretion triglyceride transport chylomicron assembly very-low-density lipoprotein particle assembly lipoprotein metabolic process cholesterol homeostasis Sources:Amigo / QuickGO
OrthologsSpeciesHuman MouseEntrez454717777EnsemblENSG00000138823ENSMUSG00000028158UniProtP55157O08601RefSeq (mRNA)NM_001300785NM_000253NM_001386140NM_001163457NM_008642NM_001355051NM_001355052RefSeq (protein)NP_000244NP_001287714NP_001156929NP_032668NP_001341980NP_001341981Location (UCSC)Chr 4: 99.56 – 99.62 MbChr 3: 137.8 – 137.85 MbPubMed search[3][4]
Wikidata
View/Edit HumanView/Edit Mouse

Microsomal triglyceride transfer protein large subunit is a protein that in humans is encoded by the MTTP, also known as MTP, gene.[5][6]

MTTP encodes the large subunit of the heterodimeric microsomal triglyceride transfer protein (MTP). Protein disulfide isomerase (PDI) completes the heterodimeric MTP, which has been shown to play a central role in lipoprotein assembly. Mutations in MTTP can cause abetalipoproteinemia.[6]

Apolipoprotein B48 on chylomicra and Apolipoprotein B100 on LDL, IDL, and VLDL are important for MTP binding.[_citation needed_]

MTP adds triglycerides to nascent chylomicrons in the intestine, and to VLDL in the liver.[7]

The large subunit of MTP, also known as the alpha subunit, contains an N-terminal half beta barrel, an alpha helix and a C-terminal lipid binding site that lies between two beta pleated sheets. It is a member of the large lipid transfer protein family, like apolipoprotein B (apo B), with which it interacts, but unlike apo B, it is not secreted. The heterodimer is instead retained in the endoplasmic reticulum due to the presence of a C-terminal KDEL motif on the PDI beta subunit.[8]

Interactive pathway map

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Click on genes, proteins and metabolites below to link to respective articles. [§ 1]

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Statin_Pathway_WP430

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Statin_Pathway_WP430

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  1. ^ The interactive pathway map can be edited at WikiPathways: "Statin_Pathway_WP430".

Drugs that inhibit MTTP prevent the assembly of apo B-containing lipoproteins thus inhibiting the synthesis of chylomicrons and VLDL and leading to decrease in plasma levels of LDL-C.

  1. ^ a b c GRCh38: Ensembl release 89: ENSG00000138823 – Ensembl, May 2017
  2. ^ a b c GRCm38: Ensembl release 89: ENSMUSG00000028158 – Ensembl, May 2017
  3. ^ "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  4. ^ "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  5. ^ Shoulders CC, Brett DJ, Bayliss JD, Narcisi TM, Jarmuz A, Grantham TT, Leoni PR, Bhattacharya S, Pease RJ, Cullen PM, et al. (Mar 1994). "Abetalipoproteinemia is caused by defects of the gene encoding the 97 kDa subunit of a microsomal triglyceride transfer protein". Hum Mol Genet. 2 (12): 2109–16. doi:10.1093/hmg/2.12.2109. PMID 8111381.
  6. ^ a b "Entrez Gene: MTTP microsomal triglyceride transfer protein".
  7. ^ Katzung BG (2018). Basic & Clinical Pharmacology (14th ed.). McGraw-Hill Education. p. 638. ISBN 978-1-259-64115-2.
  8. ^ Biterova EI, Isupov MN, Keegan RM, Lebedev AA, Sohail AA, Liaquat I, Alanen HI, Ruddock LW (2019). "The crystal structure of human microsomal triglyceride transfer protein". Proceedings of the National Academy of Sciences of the United States of America. 116 (35): 17251–17260. Bibcode:2019PNAS..11617251B. doi:10.1073/pnas.1903029116. PMC 6717300. PMID 31395737.