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Posttranslational processing of the LDL receptor and its genetic disruption in familial hypercholesterolemia.
Abstract
Synthesis of the low density lipoprotein (LDL) receptor was studied by incubation of cultured human fibroblasts with 35S-methionine followed by immunoprecipitation with a monoclonal antireceptor antibody. The receptor was synthesized as a precursor with an apparent molecular weight of 120 kilodaltons (kd) that was converted to a mature form of 160 kd. This novel form of processing occurred 15-30 min after synthesis and did not appear to be due to the simple addition of N-linked oligosaccharide chains. Fibroblasts from a child with the phenotype of homozygous familial hypercholesterolemia showed a disruption in receptor processing. This child has two different mutant alleles at the LDL receptor locus. One allele, inherited from his heterozygous mother, produces an abnormal 120 kd protein that cannot be processed to the mature 160 kd form. The other allele, inherited from his heterozygous father, produces a receptor that is synthesized as an elongated 170 kd precursor which undergoes a 40 kd increase in molecular weight to form an abnormally large receptor of 210 kd.
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The translation of LDLR mRNA yields a 120 kDa protein that is post-translationally modified in the Golgi apparatus into the mature, 160 kDa LDLR.
“The translation of LDLR mRNA yields a 120 kDa protein that is post-translationally modified in the Golgi apparatus into the mature, 160 kDa LDLR [20], [21].”
From LDLR Expression and Localization Are Altered in Mouse and Human Cell Culture Models of Alzheimer's Disease (2010) · cites this paper as [20]
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Research works printed but not linked: 22
- Asquith, 1974, Isopeptide crosslinks: their occurrence and importance in protein structure, Angew. Chem., 13, 514, 10.1002/anie.197405141 · DOI
- Basu, 1981, Monensin interrupts the recycling of low density lipoprotein receptors in human fibroblasts, Cell, 24, 493, 10.1016/0092-8674(81)90340-8 · DOI · Paper page
- Beisiegel, 1981, Monoclonal antibodies to the low density lipoprotein receptor as probes for study of receptor-mediated endocytosis and the genetics of familial hypercholesterolemia, J. Biol. Chem., 256, 11923, 10.1016/S0021-9258(19)68494-2 · DOI · Paper page
- Beisiegel, 1982, Immunoblot analysis of low density lipoprotein receptors in fibroblasts from subjects with familial hypercholesterolemia, J. Biol. Chem., 10.1016/S0021-9258(18)33635-4 · DOI
- Folk, 1977, The ϵ-(γ-glutamyl)lysine crosslink and the catalytic role of transglutaminases, Adv. Protein. Chem., 31, 1, 10.1016/S0065-3233(08)60217-X · DOI
- Goldstein, 1979, The LDL receptor locus and the genetics of familial hypercholesterolemia, Ann. Rev. Genet., 13, 259, 10.1146/annurev.ge.13.120179.001355 · DOI · Paper page
- Goldstein, 1976, Release of low density lipoprotein from its cell surface receptor by sulfated glycosaminoglycans, Cell, 7, 85, 10.1016/0092-8674(76)90258-0 · DOI
- Goldstein, 1979, Coated pits, coated vesicles, and receptor-mediated endocytosis, Nature, 279, 679, 10.1038/279679a0 · DOI · Paper page
- Green, 1981, Passage of viral membrane proteins through the Golgi complex, J. Mol. Biol., 152, 663, 10.1016/0022-2836(81)90122-4 · DOI
- Katz, 1977, Membrane assembly: synthesis and intracellular processing of the vesicular stomatitis viral glycoprotein, J. Supramol. Struct., 7, 353, 10.1002/jss.400070308 · DOI
- Krangel, 1979, Assembly and maturation of HLA-A and HLA-B antigens in vivo, Cell, 18, 979, 10.1016/0092-8674(79)90210-1 · DOI
- Laskey, 1980, The use of intensifying screens or organic scintillators for visualizing radioactive molecules resolved by gel electrophoresis, Meth. Enzymol., 65, 363, 10.1016/S0076-6879(80)65047-2 · DOI
- Lowry, 1951, Protein measurement with the folin phenol reagent, J. Biol. Chem., 193, 265, 10.1016/S0021-9258(19)52451-6 · DOI
- McConahey, 1966, A method of trace iodination of proteins for immunologic studies, Int. Arch. Allergy, 29, 185, 10.1159/000229699 · DOI
- O'Farrell, 1975, High resolution two-dimensional electrophoresis of proteins, J. Biol. Chem., 250, 4007, 10.1016/S0021-9258(19)41496-8 · DOI
- Omary, 1981, Biosynthesis of the human transferrin receptor in cultured cells, J. Biol. Chem., 256, 12888, 10.1016/S0021-9258(18)42979-1 · DOI
- Owen, 1980, Biosynthesis of HLA-A and HLA-B antigens in vivo, J. Biol. Chem., 255, 9678, 10.1016/S0021-9258(18)43446-1 · DOI
- Rothman, 1981, The Golgi apparatus: two organelles in tandem, Science, 213, 1212, 10.1126/science.7268428 · DOI
- Schneider, 1980, Partial purification and characterization of the low density lipoprotein receptor from bovine adrenal cortex, J. Biol. Chem., 255, 11442, 10.1016/S0021-9258(19)70311-1 · DOI
- Schneider, 1982, Purification of the low density lipoprotein receptor, an acidic glycoprotein of 164,000 molecular weight, J. Biol. Chem., 257, 2664, 10.1016/S0021-9258(18)34975-5 · DOI · Paper page
- Segrest, 1972, Molecular weight determination of glycoproteins by polyacrylamide gel electrophoresis in sodium dodecyl sulfate, Meth. Enzymol., 28B, 54, 10.1016/0076-6879(72)28007-7 · DOI
- Terhorst, 1981, Biochemical studies of the human thymocyte cell-surface antigens T6, T9 and T10, Cell, 23, 771, 10.1016/0092-8674(81)90441-4 · DOI
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Metadata
- Authors
- Tolleshaug, H., Goldstein, J. L., Schneider, W. J., & Brown, M. S.
- Venue
- Cell
- Publisher
- Elsevier BV
- Published
- 1982-10
- Volume
- 30
- Issue
- 3
- Pages
- 715-724
- ISSN
- 0092-8674
- Cited by
- 186
- References
- 0
- Language
- en
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