Microsomal triglyceride transfer protein large subunit

Definition:

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).

References:

[1]. E F Rehberg, et al. A novel abetalipoproteinemia genotype. Identification of a missense mutation in the 97-kDa subunit of the microsomal triglyceride transfer protein that prevents complex formation with protein disulfide isomerase. J Biol Chem. 1996 Nov 22;271(47):29945-52. [Content Brief]

[2]. Sharon A Miller, et al. Novel missense MTTP gene mutations causing abetalipoproteinemia. Biochim Biophys Acta. 2014 Oct;1842(10):1548-54. [Content Brief]

[3]. H Jamil, et al. An inhibitor of the microsomal triglyceride transfer protein inhibits apoB secretion from HepG2 cells. Proc Natl Acad Sci U S A. 1996 Oct 15;93(21):11991-5. [Content Brief]

[4]. Mathilde Di Filippo, et al. Molecular and functional analysis of two new MTTP gene mutations in an atypical case of abetalipoproteinemia. J Lipid Res. 2012 Mar;53(3):548-555. [Content Brief]

[5]. Paul Rava, et al. Transfer of cholesteryl esters and phospholipids as well as net deposition by microsomal triglyceride transfer protein. J Lipid Res. 2005 Aug;46(8):1779-85. [Content Brief]

[6]. Irani Khatun, et al. Loss of both phospholipid and triglyceride transfer activities of microsomal triglyceride transfer protein in abetalipoproteinemia. J Lipid Res. 2013 Jun;54(6):1541-1549. [Content Brief]

[7]. Paul Rava, et al. Phospholipid transfer activity of microsomal triacylglycerol transfer protein is sufficient for the assembly and secretion of apolipoprotein B lipoproteins. J Biol Chem. 2006 Apr 21;281(16):11019-27. [Content Brief]

[8]. Meghan T Walsh, et al. Novel Abetalipoproteinemia Missense Mutation Highlights the Importance of the N-Terminal β-Barrel in Microsomal Triglyceride Transfer Protein Function. Circ Cardiovasc Genet. 2015 Oct;8(5):677-87. [Content Brief]

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