| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
|
|
|
|||||||
|
|||||||||
Journal of Lipid Research, Vol 37, 1309-1315, Copyright © 1996 by Lipid Research, Inc.
EZ Du, SL Wang, HJ Kayden, R Sokol, LK Curtiss and RA Davis
Abetalipoproteinemia (ABL) is an autosomal recessive disease characterized
by the inability of the liver and intestine to secrete apolipoprotein B
(apoB). Mutations in the microsomal triglyceride transfer protein (MTP)
gene, but not the apoB gene, are responsible for the ABL phenotype. It is
not clear how loss of MTP in ABL patients leads to a complete, but
specific, block in the secretion of apoB. It is to this question that our
work is directed. In cultured cells lacking MTP, translocation of apoB is
completely arrested, leading to the hypothesis that apoB requires MTP in
order to completely enter the lumen of the endoplasmic reticulum, the site
of lipoprotein assembly. We examined this hypothesis by determining the
presence in plasma of distinct N-terminal apoB peptides, produced
exclusively from translocation arrested apoB, in the plasma of six ABL
patients and six normal subjects. The data show that N-terminal apoB
peptides are present in the plasma of six ABL patients, whereas intact
apoB-100 was barely detectable. Moreover, the plasma of all six ABL
patients displayed a 2000-fold increase in the amount of an 85 kDa
N-terminal apoB peptide relative to apoB-100. These data provide the first
in vivo data supporting the essential role that MTP plays in apoB
translocation. In normal humans, varied expression of MTP may be
responsible for the post-transcriptional regulation of apoB secretion.
ARTICLES
Translocation of apolipoprotein B across the endoplasmic reticulum is blocked in abetalipoproteinemia
Mammalian Cell and Molecular Biology Laboratory, San Diego State University, CA 92182, USA.
CiteULike 
Complore 
Connotea 
Del.icio.us 
Digg 
Reddit 
Technorati What's this?
This article has been cited by other articles:
|
|
 |
|
  N. Dashti, M. Manchekar, Y. Liu, Z. Sun, and J. P. Segrest Microsomal Triglyceride Transfer Protein Activity Is Not Required for the Initiation of Apolipoprotein B-containing Lipoprotein Assembly in McA-RH7777 Cells J. Biol. Chem., September 28, 2007; 282(39): 28597 - 28608. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 C.-M. Li, B. H. Chung, J. B. Presley, G. Malek, X. Zhang, N. Dashti, L. Li, J. Chen, K. Bradley, H. S. Kruth, et al. Lipoprotein-like Particles and Cholesteryl Esters in Human Bruch's Membrane: Initial Characterization Invest. Ophthalmol. Vis. Sci., July 1, 2005; 46(7): 2576 - 2586. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 P. E. Richardson, M. Manchekar, N. Dashti, M. K. Jones, A. Beigneux, S. G. Young, S. C. Harvey, and J. P. Segrest Assembly of Lipoprotein Particles Containing Apolipoprotein-B: Structural Model for the Nascent Lipoprotein Particle Biophys. J., April 1, 2005; 88(4): 2789 - 2800. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S. Kang, N. J. Spann, T. Y. Hui, and R. A. Davis ARP-1/COUP-TF II Determines Hepatoma Phenotype by Acting as Both a Transcriptional Repressor of Microsomal Triglyceride Transfer Protein and an Inducer of CYP7A1 J. Biol. Chem., August 15, 2003; 278(33): 30478 - 30486. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 W. Liao, T. Y. Hui, S. G. Young, and R. A. Davis Blocking microsomal triglyceride transfer protein interferes with apoB secretion without causing retention or stress in the ER J. Lipid Res., May 1, 2003; 44(5): 978 - 985. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. Kulinski, S. Rustaeus, and J. E. Vance Microsomal Triacylglycerol Transfer Protein Is Required for Lumenal Accretion of Triacylglycerol Not Associated with ApoB, as Well as for ApoB Lipidation J. Biol. Chem., August 23, 2002; 277(35): 31516 - 31525. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
E. A. Fisher and H. N. Ginsberg Complexity in the Secretory Pathway: The Assembly and Secretion of Apolipoprotein B-containing Lipoproteins J. Biol. Chem., May 10, 2002; 277(20): 17377 - 17380. [Full Text] [PDF]
|
|
|
|
 |
|
 R. A. Davis and T. Y. Hui 2000 George Lyman Duff Memorial Lecture : Atherosclerosis Is a Liver Disease of the Heart Arterioscler. Thromb. Vasc. Biol., June 1, 2001; 21(6): 887 - 898. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
Z. Li, Y. Kako, L. Pang, M. W. Freeman, J. M. Glick, X. Wang, and I. J. Goldberg Effects of overexpression of the amino-terminal fragment of apolipoprotein B on apolipoprotein B and lipoprotein production J. Lipid Res., December 1, 2000; 41(12): 1912 - 1920. [Abstract] [Full Text]
|
|
|
|
|
|
I. J. Cartwright, D. Plonné, and J. A. Higgins Intracellular events in the assembly of chylomicrons in rabbit enterocytes J. Lipid Res., November 1, 2000; 41(11): 1728 - 1739. [Abstract] [Full Text]
|
|
|
|
|
|
X. F. Huang and G. S. Shelness Efficient glycosylation site utilization by intracellular apolipoprotein B: implications for proteasomal degradation J. Lipid Res., December 1, 1999; 40(12): 2212 - 2222. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 J. R. Burnett, L. J. Wilcox, D. E. Telford, S. J. Kleinstiver, P. H. R. Barrett, R. S. Newton, and M. W. Huff The Magnitude of Decrease in Hepatic Very Low Density Lipoprotein Apolipoprotein B Secretion Is Determined by the Extent of 3-Hydroxy-3-Methylglutaryl Coenzyme A Reductase Inhibition in Miniature Pigs Endocrinology, November 1, 1999; 140(11): 5293 - 5302. [Abstract] [Full Text]
|
|
|
|
 |
|
 F. M. Riches, G. F. Watts, J. Hua, G. R. Stewart, R. P. Naoumova, and P. H. R. Barrett Reduction in Visceral Adipose Tissue Is Associated with Improvement in Apolipoprotein B-100 Metabolism in Obese Men J. Clin. Endocrinol. Metab., August 1, 1999; 84(8): 2854 - 2861. [Abstract] [Full Text]
|
|
|
|
|
|
J. R. Burnett, L. J. Wilcox, D. E. Telford, S. J. Kleinstiver, P. H. R. Barrett, R. S. Newton, and M. W. Huff Inhibition of ACAT by avasimibe decreases both VLDL and LDL apolipoprotein B production in miniature pigs J. Lipid Res., July 1, 1999; 40(7): 1317 - 1328. [Abstract] [Full Text]
|
|
|
|
|
|
J. F. Fleming, G. M. Spitsen, T. Y. Hui, L. Olivier, E. Z. Du, M. Raabe, and R. A. Davis Chinese Hamster Ovary Cells Require the Coexpression of Microsomal Triglyceride Transfer Protein and Cholesterol 7alpha -Hydroxylase for the Assembly and Secretion of Apolipoprotein B-containing Lipoproteins J. Biol. Chem., April 2, 1999; 274(14): 9509 - 9514. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
L. J. Wilcox, P. H. R. Barrett, R. S. Newton, and M. W. Huff ApoB100 Secretion From HepG2 Cells is Decreased by the ACAT Inhibitor CI-1011 : An Effect Associated With Enhanced Intracellular Degradation of ApoB Arterioscler. Thromb. Vasc. Biol., April 1, 1999; 19(4): 939 - 949. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. Theriault, Q. Wang, A. Gapor, and K. Adeli Effects of {gamma}-Tocotrienol on ApoB Synthesis, Degradation, and Secretion in HepG2 Cells Arterioscler. Thromb. Vasc. Biol., March 1, 1999; 19(3): 704 - 712. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 G. S. Shelness, M. F. Ingram, X. F. Huang, and J. A. DeLozier Apolipoprotein B in the Rough Endoplasmic Reticulum: Translation, Translocation and the Initiation of Lipoprotein Assembly J. Nutr., February 1, 1999; 129(2): 456 - 456. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
E. Z. Du, J. F. Fleming, S.-L. Wang, G. M. Spitsen, and R. A. Davis Translocation-arrested Apolipoprotein B Evades Proteasome Degradation via a Sterol-sensitive Block in Ubiquitin Conjugation J. Biol. Chem., January 15, 1999; 274(3): 1856 - 1862. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J.-s. Liang, X. Wu, H. Jiang, M. Zhou, H. Yang, P. Angkeow, L.-S. Huang, S. L. Sturley, and H. Ginsberg Translocation Efficiency, Susceptibility to Proteasomal Degradation, and Lipid Responsiveness of Apolipoprotein B Are Determined by the Presence of beta Sheet Domains J. Biol. Chem., December 25, 1998; 273(52): 35216 - 35221. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
M. Zhou, E. A. Fisher, and H. N. Ginsberg Regulated Co-translational Ubiquitination of Apolipoprotein B100. A NEW PARADIGM FOR PROTEASOMAL DEGRADATION OF A SECRETORY PROTEIN J. Biol. Chem., September 18, 1998; 273(38): 24649 - 24653. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
F. M. Riches, G. F. Watts, F. M. van Bockxmeer, J. Hua, S. Song, S. E. Humphries, and P. J. Talmud Apolipoprotein B signal peptide and apolipoprotein E genotypes as determinants of the hepatic secretion of VLDL apoB in obese men J. Lipid Res., September 1, 1998; 39(9): 1752 - 1758. [Abstract] [Full Text]
|
|
|
|
 |
|
 M. Raabe, L. M. Flynn, C. H. Zlot, J. S. Wong, M. M. Veniant, R. L. Hamilton, and S. G. Young Knockout of the abetalipoproteinemia gene in mice: Reduced lipoprotein secretion in heterozygotes and embryonic lethality in homozygotes PNAS, July 21, 1998; 95(15): 8686 - 8691. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. E. Rusiñol, R. S. Hegde, S. L. Chuck, V. R. Lingappa, and J. E. Vance Translocational pausing of apolipoprotein B can be regulated by membrane lipid composition J. Lipid Res., June 1, 1998; 39(6): 1287 - 1294. [Abstract] [Full Text]
|
|
|
|
 |
|
 P. S. Kim and P. Arvan Endocrinopathies in the Family of Endoplasmic Reticulum (ER) Storage Diseases: Disorders of Protein Trafficking and the Role of ER Molecular Chaperones Endocr. Rev., April 1, 1998; 19(2): 173 - 202. [Abstract] [Full Text]
|
|
|
|
|
|
J. R. Burnett, L. J. Wilcox, D. E. Telford, S. J. Kleinstiver, P. H. R. Barrett, R. S. Newton, and M. W. Huff Inhibition of HMG-CoA Reductase by Atorvastatin Decreases Both VLDL and LDL Apolipoprotein B Production in Miniature Pigs Arterioscler. Thromb. Vasc. Biol., November 1, 1997; 17(11): 2589 - 2600. [Abstract] [Full Text]
|
|
|
|
|
|
L. Wang, D. G. Fast, and A. D. Attie The Enzymatic and Non-enzymatic Roles of Protein-disulfide Isomerase in Apolipoprotein B Secretion J. Biol. Chem., October 31, 1997; 272(44): 27644 - 27651. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. E. Rusinol, H. Jamil, and J. E. Vance In Vitro Reconstitution of Assembly of Apolipoprotein B48-containing Lipoproteins J. Biol. Chem., March 21, 1997; 272(12): 8019 - 8025. [Abstract] [Full Text] [PDF]
|
|
| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
| All ASBMB Journals | Journal of Biological Chemistry |
| Molecular and Cellular Proteomics | ASBMB Today |
