Endoplasmic reticulum quality control in lipoprotein metabolism

Aldahmesh, 2013, Mutations in LRPAP1 are associated with severe myopia in Humans, Am. J. Hum. Genet., 93, 313, 10.1016/j.ajhg.2013.06.002 Andersen, 2003, Hepatic lipase mutations, elevated high-density lipoprotein cholesterol, and increased risk of ischemic heart disease: the Copenhagen City Heart Study, J. Am. Coll. Cardiol., 41, 1972, 10.1016/S0735-1097(03)00407-8 Arora, 2019, Structure of lipoprotein lipase in complex with GPIHBP1, Proc. Natl. Acad. Sci., 116, 10360, 10.1073/pnas.1820171116 Atkins, 1998, Calcium is essential for the structural integrity of the cysteine-rich, ligand-binding repeat of the low-density lipoprotein receptor, Biochemistry, 37, 1662, 10.1021/bi972529n Babilonia-Rosa, 2014, Purification, cellular levels, and functional domains of lipase maturation factor 1, Biochem. Biophys. Res. Commun., 450, 423, 10.1016/j.bbrc.2014.05.136 Barakizou, 2016, Abetalipoproteinemia: a novel mutation of microsomal triglyceride transfer protein (MTP) gene in a young Tunisian patient, Egypt J. Med. Hum. Genet., 17, 251, 10.1016/j.ejmhg.2015.12.003 Ben-Zeev, 2004, Maturation of hepatic lipase: formation of functional enzyme in the endoplasmic reticulum is the rate-limiting step in its secretion, J. Biol. Chem., 279, 6171, 10.1074/jbc.M310051200 Ben-Zeev, 1992, Maturation of lipoprotein lipase. Expression of full catalytic activity requires glucose trimming but not translocation to the cis-Golgi compartment, J. Biol. Chem., 267, 6219, 10.1016/S0021-9258(18)42684-1 Biterova, 2019, The crystal structure of human microsomal triglyceride transfer protein, Proc. Natl. Acad. Sci. U. S. A., 10.1073/pnas.1903029116 Brown, 1976, Receptor-mediated control of cholesterol metabolism, Science, 191, 10.1126/science.174194 Bu, 1995, 39 kDa receptor-associated protein is an ER resident protein and molecular chaperone for LDL receptor-related protein, EMBO J., 14, 2269, 10.1002/j.1460-2075.1995.tb07221.x Bulleid, 1988, Defective co-translational formation of disulphide bonds in protein disulphide-isomerase-deficient microsomes, Nature, 335, 649, 10.1038/335649a0 Burch, 2000, Disulfide bonds are required for folding and secretion of apolipoprotein B regardless of its lipidation state, J. Biol. Chem., 275, 16267, 10.1074/jbc.M000446200 Burnett, 2003, A novel nontruncating APOB gene mutation, R463W, causes familial hypobetalipoproteinemia, J. Biol. Chem., 278, 13442, 10.1074/jbc.M300235200 Burnett, 2007, Missense mutations in APOB within the betaalpha1 domain of human APOB-100 result in impaired secretion of ApoB and ApoB-containing lipoproteins in familial hypobetalipoproteinemia, J. Biol. Chem., 282, 24270, 10.1074/jbc.M702442200 Buscá, 1996, The mutation gly142 → glu in human lipoprotein lipase produces a missorted protein that is diverted to lysosomes, J. Biol. Chem., 271, 2139, 10.1074/jbc.271.4.2139 Buscá, 1995, Absence of N-glycosylation at asparagine 43 in human lipoprotein lipase induces its accumulation in the rough endoplasmic reticulum and alters this cellular compartment, J. Lipid Res., 36, 939, 10.1016/S0022-2275(20)39852-7 Carmichael, 1977, Purification and characterization of a thiol:protein disulfide oxidoreductase from bovine liver, J. Biol. Chem., 252, 7163, 10.1016/S0021-9258(19)66949-8 Chen, 1998, Calnexin and other factors that alter translocation affect the rapid binding of ubiquitin to ApoB in the Sec61 complex, J. Biol. Chem., 273, 11887, 10.1074/jbc.273.19.11887 Chen, 2002, Hypobetalipoproteinemic mice with a targeted apolipoprotein (Apo) B-27.6-specifying mutation: in vivo evidence for an important role of amino acids 1254-1744 of ApoB in lipid transport and metabolism of the apoB-containing lipoprotein, J. Biol. Chem., 277, 14135, 10.1074/jbc.M200617200 Chora, 2018, Analysis of publicly available LDLR, APOB, and PCSK9 variants associated with familial hypercholesterolemia: application of ACMG guidelines and implications for familial hypercholesterolemia diagnosis, Genet. Med., 20, 591, 10.1038/gim.2017.151 Culi, 2003, Boca, an endoplasmic reticulum protein required for wingless signaling and trafficking of LDL receptor family members in Drosophila, Cell, 112, 343, 10.1016/S0092-8674(02)01279-5 Culi, 2004, Boca-dependent maturation of β-propeller/EGF modules in low-density lipoprotein receptor proteins, EMBO J., 23, 1372, 10.1038/sj.emboj.7600132 Dieckmann, 2010, Lipoprotein receptors-an evolutionarily ancient multifunctional receptor family, Biol. Chem., 391, 1341, 10.1515/bc.2010.129 Doolittle, 2009, Hepatic lipase maturation: a partial proteome of interacting factors, J. Lipid Res., 50, 1173, 10.1194/jlr.M800603-JLR200 Ehrhardt, 2014, Embryonic viability, lipase deficiency, hypertriglyceridemia and neonatal lethality in a novel LMF1-deficient mouse model, Nutr. Metab., 11, 1, 10.1186/1743-7075-11-37 Ellgaard, 2003, Quality control in the endoplasmic reticulum, Nat. Rev. Mol. Cell Biol., 4, 181, 10.1038/nrm1052 Ellgaard, 1999, Setting the standards: quality control in the secretory pathway, Science, 286, 1882, 10.1126/science.286.5446.1882 Esser, 1988, Mutational analysis of the ligand binding domain of the low density lipoprotein receptor, J. Biol. Chem., 263, 13282, 10.1016/S0021-9258(18)37702-0 Farese, 1995, Knockout of the mouse apolipoprotein B gene results in embryonic lethality in homozygotes and protection against diet-induced hypercholesterolemia in heterozygotes, Proc. Natl. Acad. Sci. U. S. A., 92, 1774, 10.1073/pnas.92.5.1774 Fass, 1997, Molecular basis of familial hypercholesterolaemia from structure of LDL receptor module, Nature, 388, 691, 10.1038/41798 Fisher, 2006, Structure of an LDLR-RAP Complex reveals a general mode for ligand recognition by lipoprotein receptors, Mol. Cell, 22, 277, 10.1016/j.molcel.2006.02.021 Fisher, 2001, The triple threat to nascent apolipoprotein B, J. Biol. Chem., 276, 27855, 10.1074/jbc.M008885200 Fisher, 1997, Common variation in the lipoprotein lipase gene: effects on plasma lipids and risk of atherosclerosis, Atherosclerosis, 135, 145, 10.1016/S0021-9150(97)00199-8 Glickman, 1986, Apolipoprotein B synthesis by human liver and intestine in vitro, Proc. Natl. Acad. Sci., 83, 5296, 10.1073/pnas.83.14.5296 Gonzalez-Teuber, 2019, Small molecules to improve ER proteostasis in disease, Trends Pharmacol. Sci., 10.1016/j.tips.2019.07.003 Gordon, 1994, Secretion of apolipoprotein B-containing lipoproteins from HeLa cells is dependent on expression of the microsomal triglyceride transfer protein and is regulated by lipid availability, Proc. Natl. Acad. Sci., 91, 7628, 10.1073/pnas.91.16.7628 Griffon, 2009, Identification of the active form of endothelial lipase, a homodimer in a head-to-tail conformation, J. Biol. Chem., 284, 23322, 10.1074/jbc.M109.037002 Heiss, 1980, Lipoprotein-cholesterol distributions in selected North American populations: the lipid research clinics program prevalence study, Circulation, 61, 302, 10.1161/01.CIR.61.2.302 Herz, 1991, 39-kDa protein modulates binding of ligands to low density lipoprotein receptor-related protein/alpha 2-macroglobulin receptor, J. Biol. Chem., 266, 21232, 10.1016/S0021-9258(18)54845-6 Hobbs, 1992, Molecular genetics of the LDL receptor gene in familial hypercholesterolemia, Hum. Mutat., 1, 445, 10.1002/humu.1380010602 Homanics, 1995, Mild dyslipidemia in mice following targeted inactivation of the hepatic lipase gene, J. Biol. Chem., 270, 2974, 10.1074/jbc.270.7.2974 Huang, 1997, Identification of cysteine pairs within the amino-terminal 5% of apolipoprotein B essential for hepatic lipoprotein assembly and secretion, J. Biol. Chem., 272, 31872, 10.1074/jbc.272.50.31872 Hussain, 1997, Apolipoprotein B binding to microsomal triglyceride transfer protein decreases with increases in length and lipidation:  implications in lipoprotein biosynthesis, Biochem, 36, 13060, 10.1021/bi971395a Iijima, 1998, Expression and characterization of a very low density lipoprotein receptor variant lacking the O-linked sugar region generated by alternative splicing, J. Biochem., 124, 747, 10.1093/oxfordjournals.jbchem.a022175 Jamil, 1996, An inhibitor of the microsomal triglyceride transfer protein inhibits apoB secretion from HepG2 cells, Proc. Natl. Acad. Sci., 93, 10.1073/pnas.93.21.11991 Jansens, 2002, Coordinated nonvectorial folding in a newly synthesized multidomain protein, Science, 298, 2401, 10.1126/science.1078376 Jeon, 2001, Implications for familial hypercholesterolemia from the structure of the LDL receptor YWTD-EGF domain pair, Nat. Struct. Biol., 8, 499, 10.1038/88556 Jiang, 2006, Defining lipid-interacting domains in the N-terminal region of apolipoprotein B, Biochemistry, 45, 11799, 10.1021/bi060600w Jørgensen, 2000, Grp78 is involved in retention of mutant low density lipoprotein receptor protein in the endoplasmic reticulum, J. Biol. Chem., 275, 33861, 10.1074/jbc.M004663200 Kane, 1980, Heterogeneity of apolipoprotein B: isolation of a new species from human chylomicrons, Proc. Natl. Acad. Sci., 77, 2465, 10.1073/pnas.77.5.2465 Koduri, 2001, Folding determinants of LDL receptor type A modules, Biochemistry, 40, 12801, 10.1021/bi011344k Koduri, 2007, Requirement for natively unstructured regions of mesoderm development candidate 2 in promoting low-density lipoprotein receptor-related protein 6 maturation, Biochemistry, 46, 6570, 10.1021/bi700049g Köhler, 2011, The structure of MESD45-184 brings light into the mechanism of LDLR family folding, Structure, 19, 337, 10.1016/j.str.2010.12.022 Kozarsky, 1988, Use of a mutant cell line to study the kinetics and function of O-linked glycosylation of low density lipoprotein receptors, Proc. Natl. Acad. Sci., 85, 4335, 10.1073/pnas.85.12.4335 Kulinski, 2002, Microsomal triacylglycerol transfer protein is required for lumenal accretion of triacylglycerol not associated with ApoB, as well as for ApoB lipidation, J. Biol. Chem., 277, 31516, 10.1074/jbc.M202015200 Kurniawan, 2001, NMR structure and backbone dynamics of a concatemer of epidermal growth factor homology modules of the human low-density lipoprotein receptor, J. Mol. Biol., 311, 341, 10.1006/jmbi.2001.4867 Lee, 2006, RAP uses a histidine switch to regulate its interaction with LRP in the ER and Golgi, Mol. Cell, 22, 423, 10.1016/j.molcel.2006.04.011 Leiserson, 2013, Simultaneous identification of multiple driver pathways in cancer, PLoS Comput. Biol., 9, 10.1371/journal.pcbi.1003054 Li, 2002, Receptor-associated protein facilitates proper folding and maturation of the low-density lipoprotein receptor and its class 2 mutants, Biochemistry, 41, 4921, 10.1021/bi011894i Li, 2004, Degradation of the LDL receptor class 2 mutants is mediated by a proteasome-dependent pathway, J. Lipid Res., 45, 1084, 10.1194/jlr.M300482-JLR200 Liang, 2003, Overexpression of the tumor autocrine motility factor receptor Gp78, a ubiquitin protein ligase, results in increased ubiquitinylation and decreased secretion of apolipoprotein B100 in HepG2 cells, J. Biol. Chem., 278, 23984, 10.1074/jbc.M302683200 Luo, 2017, Identification of a molecular signaling gene-gene regulatory network between GWAS susceptibility genes ADTRP and MIA3/TANGO1 for coronary artery disease, Biochim. Biophys. Acta (BBA) - Mol. Basis Dis., 1863, 1640, 10.1016/j.bbadis.2017.03.010 Lyumkis, 2019, Challenges and opportunities in cryo-EM single-particle analysis, J. Biol. Chem., 294, 5181, 10.1074/jbc.REV118.005602 MacArthur, 2014, Guidelines for investigating causality of sequence variants in human disease, Nature, 508, 469, 10.1038/nature13127 Marks, 2003, A review on the diagnosis, natural history, and treatment of familial hypercholesterolaemia, Atherosclerosis, 168, 1, 10.1016/S0021-9150(02)00330-1 Marques-Vidal, 1991, Hepatic lipase-mediated hydrolysis versus liver uptake of HDL phospholipids and triacylglycerols by the perfused rat liver, Biochim. Biophys. Acta Lipids Lipid Metab., 1082, 185, 10.1016/0005-2760(91)90193-L Michalak, 2009, Calreticulin, a multi-process calcium-buffering chaperone of the endoplasmic reticulum, Biochem. J., 417, 651, 10.1042/BJ20081847 Miller, 2004, Role of N-linked glycosylation in the secretion and activity of endothelial lipase, J. Lipid Res., 45, 2080, 10.1194/jlr.M400162-JLR200 Murthy, 1996, Molecular pathobiology of the human lipoprotein lipase gene, Pharmacol. Ther., 70, 101, 10.1016/0163-7258(96)00005-8 Musliner, 1979, Lipoprotein substrates of lipoprotein lipase and hepatic triacylglycerol lipase from human post-heparin plasma, Biochim. Biophys. Acta Mol. Cell Biol. Lipids, 575, 277, 10.1016/0005-2760(79)90029-8 Oka, 2013, ERdj5 is the ER reductase that catalyzes the removal of non-native disulfides and correct folding of the LDL receptor, Mol. Cell, 50, 793, 10.1016/j.molcel.2013.05.014 Olivecrona, 1985, Combined lipase deficiency (cld/cld) in mice, J. Biol. Chem., 260, 2552, 10.1016/S0021-9258(18)89587-4 Pan, 2008, Presecretory oxidation, aggregation, and autophagic destruction of apoprotein-B: a pathway for late-stage quality control, Proc. Natl. Acad. Sci., 105, 5862, 10.1073/pnas.0707460104 Paterniti, 1983, Combined lipase deficiency (cld): a lethal mutation on chromosome 17 of the mouse, Science, 221, 167, 10.1126/science.6857276 Patni, 2000, Genetics and dyslipidemia Péterfy, 2007, Mutations in LMF1 cause combined lipase deficiency and severe hypertriglyceridemia, Nat. Genet., 39, 1483, 10.1038/ng.2007.24 Ramasamy, 2016, Update on the molecular biology of dyslipidemias, Clin. Chim. Acta, 454, 143, 10.1016/j.cca.2015.10.033 Rehberg, 1996, 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., 271, 29945, 10.1074/jbc.271.47.29945 Roberts, 2018, Lipase maturation factor 1 affects redox homeostasis in the endoplasmic reticulum, EMBO J., 298, 2353 Rodrigues, 2016, Pathogenic classification of LPL gene variants reported to be associated with LPL deficiency, J. Clin. Lipidol., 10, 394, 10.1016/j.jacl.2015.12.015 Rudenko, 2002, Structure of the LDL receptor extracellular domain at endosomal pH, Science, 10.1126/science.1078124 Ruf, 1998, Size distributions of chylomicrons from human lymph from dynamic light scattering measurements, Eur. Biophys. J., 28, 1, 10.1007/s002490050178 Rutledge, 2009, Mechanisms targeting apolipoprotein B100 to proteasomal degradation, Arterioscler. Thromb. Vasc. Biol., 29, 579, 10.1161/ATVBAHA.108.181859 Saito, 2009, TANGO1 facilitates cargo loading at endoplasmic reticulum exit sites, Cell, 136, 891, 10.1016/j.cell.2008.12.025 Sani, 2011, Identification of a novel mutation of MTP gene in a patient with abetalipoproteinemia, Ann. Hepatol., 10, 221, 10.1016/S1665-2681(19)31573-X Santos, 2016, TANGO1 and Mia2/cTAGE5 (TALI) cooperate to export bulky pre-chylomicrons/VLDLs from the endoplasmic reticulum, J. Cell Biol., 213, 343, 10.1083/jcb.201603072 Schonfeld, 2003, Familial hypobetalipoproteinemia: a review, J. Lipid Res., 44, 878, 10.1194/jlr.R300002-JLR200 Schumaker, 1994, Apolipoprotein B and low-density lipoprotein structure: implications for biosynthesis of triglyceride-rich lipoproteins, Adv. Protein Chem., 45, 205, 10.1016/S0065-3233(08)60641-5 Segrest, 2001, Structure of apolipoprotein B-100 in low density lipoproteins, J. Lipid Res., 42, 1346, 10.1016/S0022-2275(20)30267-4 Serveaux Dancer, 2018, New rare genetic variants of LMF1 gene identified in severe hypertriglyceridemia, J. Clin. Lipidol., 12, 1244, 10.1016/j.jacl.2018.06.018 Sha, 2014, The ER-associated degradation adaptor protein Sel1L regulates LPL secretion and lipid metabolism, Cell Metabol., 20, 458, 10.1016/j.cmet.2014.06.015 Shelness, 1996, Role of intramolecular disulfide bond formation in the assembly and secretion of apolipoprotein B-100-containing lipoproteins, J. Lipid Res., 37, 408, 10.1016/S0022-2275(20)37627-6 Simha, 2009, Inherited lipodystrophies and hypertriglyceridemia, Curr. Opin. Lipidol., 20, 300, 10.1097/MOL.0b013e32832d4a33 Simsolo, 1992, Characterization of lipoprotein lipase activity, secretion, and degradation at different sites of post-translational processing in primary cultures of rat adipocytes, J. Lipid Res., 33, 1777, 10.1016/S0022-2275(20)41335-5 Sirwi, 2018, Lipid transfer proteins in the assembly of apoB-containing lipoproteins, J. Lipid Res., 59, 1094, 10.1194/jlr.R083451 Sørensen, 2005, Retention of mutant low density lipoprotein receptor in endoplasmic reticulum (ER) leads to ER stress, J. Biol. Chem., 281, 468, 10.1074/jbc.M507071200 Strom, 2015, Mutation p.L799R in the LDLR, which affects the transmembrane domain of the LDLR, prevents membrane insertion and causes secretion of the mutant LDLR, Hum. Mol. Genet., 24, 5836, 10.1093/hmg/ddv304 Strom, 2011, Characterization of residues in the cytoplasmic domain of the LDL receptor required for exit from the endoplasmic reticulum, Biochem. Biophys. Res. Commun., 415, 642, 10.1016/j.bbrc.2011.10.127 Tatu, 1999, Interaction of newly synthesized apolipoprotein B with calnexin and calreticulin requires glucose trimming in the endoplasmic reticulum, Biosci. Rep., 19, 189, 10.1023/A:1020225803282 Tran, 1998, Functional analysis of disulfide linkages clustered within the amino terminus of human apolipoprotein B, J. Biol. Chem., 273, 7244, 10.1074/jbc.273.13.7244 Trombetta, 1989, Glucosylation of glycoproteins by mammalian, plant, fungal, and trypanosomatid protozoa microsomal membranes, Biochemistry, 28, 8108, 10.1021/bi00446a022 Ushioda, 2008, ERdj5 is required as a disulfide reductase for degradation of misfolded proteins in the ER, Science, 321, 569, 10.1126/science.1159293 Usifo, 2012, Low-density lipoprotein receptor gene familial hypercholesterolemia variant database: update and pathological assessment, Ann. Hum. Genet., 387, 10.1111/j.1469-1809.2012.00724.x Vannier, 1989, Biosynthesis of lipoprotein lipase in cultured mouse adipocytes, J. Biol. Chem., 264, 13206, 10.1016/S0021-9258(18)51616-1 Vukmirica, 2002, The N-linked oligosaccharides at the amino terminus of human apoB are important for the assembly and secretion of VLDL, J. Lipid Res., 43, 1496, 10.1194/jlr.M200077-JLR200 Walsh, 2016, Structure-function analyses of microsomal triglyceride transfer protein missense mutations in abetalipoproteinemia and hypobetalipoproteinemia subjects, Biochim. Biophys. Acta Mol. Cell Biol. Lipids, 1861, 1623, 10.1016/j.bbalip.2016.07.015 Wang, 1997, The enzymatic and non-enzymatic roles of protein-disulfide isomerase in apolipoprotein B secretion, J. Biol. Chem., 272, 27644, 10.1074/jbc.272.44.27644 Wang, 2015, Identification of protein disulfide isomerase 1 as a key isomerase for disulfide bond formation in apolipoprotein B100, Mol. Biol. Cell, 26, 594, 10.1091/mbc.E14-08-1274 Wefer, 2003, Mesd encodes an LRP5/6 chaperone essential for specification of mouse embryonic polarity, Cell, 112, 355, 10.1016/S0092-8674(03)00045-X Weinstock, 1995, Severe hypertriglyceridemia, reduced high density lipoprotein, and neonatal death in lipoprotein lipase knockout mice. Mild hypertriglyceridemia with impaired very low density lipoprotein clearance in heterozygotes, J. Clin. Investig., 2555, 10.1172/JCI118319 Wetterau, 1992, Absence of microsomal triglyceride transfer protein in individuals with abetalipoproteinemia, Science, 258, 999, 10.1126/science.1439810 Wetterau, 1991, Protein disulfide isomerase appears necessary to maintain the catalytically active structure of the microsomal triglyceride transfer protein, Biochemistry, 30, 9728, 10.1021/bi00104a023 Wetterau, 1984, A triglyceride and cholesteryl ester transfer protein associated with liver microsomes, J. Biol. Chem., 259, 10863, 10.1016/S0021-9258(18)90593-4 Wetterau, 1985, Purification and characterization of microsomal triglyceride and cholesteryl ester transfer protein from bovine liver microsomes, Chem. Phys. Lipids, 38, 205, 10.1016/0009-3084(85)90068-4 Willnow, 1995, Functional expression of low density lipoprotein receptor-related protein is controlled by receptor-associated protein in vivo, Proc. Natl. Acad. Sci. U. S. A, 92, 4537, 10.1073/pnas.92.10.4537 Wittrup, 1999, Lipoprotein lipase mutations, plasma lipids and lipoproteins, and risk of ischemic heart disease: a meta-analysis, Circulation, 99, 2901, 10.1161/01.CIR.99.22.2901 Wong, 2002, The lipase gene family, J. Lipid Res., 43, 993, 10.1194/jlr.R200007-JLR200 Wu, 2018, Co-expression of novel furin-resistant LPL variants with LMF1 enhances LPL secretion and activity, JLR (J. Lipid Res.), 1 Yang, 1990, Isolation and characterization of sulfhydryl and disulfide peptides of human apolipoprotein B-100, Proc. Natl. Acad. Sci., 87, 5523, 10.1073/pnas.87.14.5523 Ye, 2009, Cideb, an ER- and lipid droplet-associated protein, mediates VLDL lipidation and maturation by Interacting with Apolipoprotein B, Cell Metabol., 9, 177, 10.1016/j.cmet.2008.12.013 Ye, 2004, A membrane protein complex mediates retro-translocation from the ER lumen into the cytosol, Nature, 429, 841, 10.1038/nature02656 Yeung, 1996, Ubiquitin-proteasome pathway mediates intracellular degradation of apolipoprotein B, Biochemistry, 35, 10.1021/bi9618777 Zhang, 2014, Cideb facilitates the lipidation of chylomicrons in the small intestine, J. Lipid Res., 55, 1279, 10.1194/jlr.M046482 Zhang, 2005, Calcium triggers folding of lipoprotein lipase into active dimers, J. Biol. Chem., 280, 42580, 10.1074/jbc.M507252200 Zhang, 2003, Calreticulin promotes folding/dimerization of human lipoprotein lipase expressed in insect cells, J. Biol. Chem., 278, 29344, 10.1074/jbc.M300455200