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Apolipoprotein B48

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Lawrence P. Aggerbeck – One of the best experts on this subject based on the ideXlab platform.

  • anderson s disease chylomicron retention disease in a japanese patient with uniparental disomy 7 and a normal sar1b gene protein coding sequence
    Orphanet Journal of Rare Diseases, 2011
    Co-Authors: Tomoo Okada, Michio Miyashita, Junji Fukuhara, Masahiko Sugitani, Takahiro Ueno, Marie Elisabeth Samsonbouma, Lawrence P. Aggerbeck

    Abstract:

    Background
    Anderson’s Disease (AD)/Chylomicron Retention Disease (CMRD) is a rare hereditary hypocholesterolemic disorder characterized by a malabsorption syndrome with steatorrhea, failure to thrive and the absence of chylomicrons and Apolipoprotein B48 post-prandially. All patients studied to date exhibit a mutation in the SAR1B gene, which codes for an essential component of the vesicular coat protein complex II (COPII) necessary for endoplasmic reticulum to Golgi transport. We describe here a patient with AD/CMRD, a normal SAR1B gene protein coding sequence and maternal uniparental disomy of chromosome 7 (matUPD7).

  • Anderson’s disease/chylomicron retention disease in a Japanese patient with uniparental disomy 7 and a normal SAR1B gene protein coding sequence
    Orphanet journal of rare diseases, 2011
    Co-Authors: Tomoo Okada, Michio Miyashita, Junji Fukuhara, Masahiko Sugitani, Takahiro Ueno, Marie-elisabeth Samson-bouma, Lawrence P. Aggerbeck

    Abstract:

    Background
    Anderson’s Disease (AD)/Chylomicron Retention Disease (CMRD) is a rare hereditary hypocholesterolemic disorder characterized by a malabsorption syndrome with steatorrhea, failure to thrive and the absence of chylomicrons and Apolipoprotein B48 post-prandially. All patients studied to date exhibit a mutation in the SAR1B gene, which codes for an essential component of the vesicular coat protein complex II (COPII) necessary for endoplasmic reticulum to Golgi transport. We describe here a patient with AD/CMRD, a normal SAR1B gene protein coding sequence and maternal uniparental disomy of chromosome 7 (matUPD7).

  • Anderson’s disease/chylomicron retention disease in a Japanese patient with uniparental disomy 7 and a normal SAR1B gene protein coding sequence.
    Orphanet Journal of Rare Diseases, 2011
    Co-Authors: Tomoo Okada, Michio Miyashita, Junji Fukuhara, Masahiko Sugitani, Takahiro Ueno, Marie-elisabeth Samson-bouma, Lawrence P. Aggerbeck

    Abstract:

    UNLABELLED: ABSTRACT: BACKGROUND: Anderson’s Disease (AD)/Chylomicron Retention Disease (CMRD) is a rare hereditary hypocholesterolemic disorder characterized by a malabsorption syndrome with steatorrhea, failure to thrive and the absence of chylomicrons and Apolipoprotein B48 post-prandially. All patients studied to date exhibit a mutation in the SAR1B gene, which codes for an essential component of the vesicular coat protein complex II (COPII) necessary for endoplasmic reticulum to Golgi transport. We describe here a patient with AD/CMRD, a normal SAR1B gene protein coding sequence and maternal uniparental disomy of chromosome 7 (matUPD7). METHODS AND RESULTS: The patient, one of two siblings of a Japanese family, had diarrhea and steatorrhea beginning at five months of age. There was a white duodenal mucosa upon endoscopy. Light and electron microscopy showed that the intestinal villi were normal but that they had lipid laden enterocytes containing accumulations of lipid droplets in the cytoplasm and lipoprotein-size particles in membrane bound structures. Although there were decreased amounts in plasma of total- and low-density lipoprotein cholesterol, Apolipoproteins AI and B and vitamin E levels, the triglycerides were normal, typical of AD/CMRD. The presence of low density lipoproteins and Apolipoprotein B in the plasma, although in decreased amounts, ruled out abetalipoproteinemia. The parents were asymptomatic with normal plasma cholesterol levels suggesting a recessive disorder and ruling out familial hypobetalipoproteinemia. Sequencing of genomic DNA showed that the 8 exons of the SAR1B gene were normal. Whole genome SNP analysis and karyotyping revealed matUPD7 with a normal karyotype. In contrast to other cases of AD/CMRD which have shown catch-up growth following vitamin supplementation and a fat restricted diet, our patient exhibits continued growth delay and other aspects of the matUPD7 and Silver-Russell Syndrome phenotypes. CONCLUSIONS: This patient with AD/CMRD has a normal SAR1B gene protein coding sequence which suggests that factors other than the SAR1B protein may be crucial for chylomicron secretion. Further, this patient exhibits matUPD7 with regions of homozygosity which might be useful for elucidating the molecular basis of the defect(s) in this individual. The results provide novel insights into the relation between phenotype and genotype in these diseases and for the mechanisms of secretion in the intestine.

John C L Mamo – One of the best experts on this subject based on the ideXlab platform.

  • increased risk of cardiovascular disease in type 1 diabetes arterial exposure to remnant lipoproteins leads to enhanced deposition of cholesterol and binding to glycated extracellular matrix proteoglycans
    Diabetic Medicine, 2011
    Co-Authors: Rabban Mangat, Donna F Vine, Jennifer E Lambert, M T Clandinin, Jenny W Su, Ye Wang, Richard R E Uwiera, Josephine M Forbes, Mark E Cooper, John C L Mamo

    Abstract:

    Aims To determine fasting and postprandial metabolism of Apolipoprotein B48 (apoB48) remnant lipoproteins in subjects with Type 1 diabetes and the relationship to progressive cardiovascular disease, and to investigate the impact of remnant lipoprotein cholesterol accumulation associated with arterial wall biglycan using a rodent model of Type 1 diabetes.Methods Normolipidaemic subjects (n = 9) with long-standing Type 1 diabetes (and advanced cardiovascular disease) and seven healthy control subjects were studied. Fasting and postprandial apoB48 concentration was determined following a sequential meal challenge. A rodent model of streptozotocin-induced diabetes was used to investigate the ex vivo retention of fluorescent-conjugated remnants. Binding of remnant lipoproteins to human recombinant biglycan was assessed in vitro.Results A significantly higher concentration of fasting plasma apoB48 remnants was observed in patients with Type 1 diabetes compared with control subjects. Patients with Type 1 diabetes exhibited a greater total plasma apoB48 area under the curve (AUC) and an increased incremental AUC following a second sequential meal compared with control subjects. The arterial retention of remnants ex vivo and associated cholesterol was increased sevenfold in Type 1 diabetes rats relative to controls. Remnants were shown to bind with significant affinity to human biglycan in vitro and a further 2.3-fold increased binding capacity was observed with glycated biglycan. Remnants were shown to colocalize with both arterial biglycan and glycated matrix proteins in the Type 1 diabetes rodent model.Conclusion Impaired metabolism of remnant lipoproteins associated with enhanced binding to proteoglycans appears to contribute to the arterial cholesterol deposition in Type 1 diabetes. Our findings support the hypothesis that impaired remnant metabolism may contribute to accelerated progression of atherosclerosis in the hyperglycaemic and insulin-deficient state. © 2010 The Authors. Diabetic Medicine

  • Prior exercise does not affect chylomicron particle number following a mixed meal of moderate fat content
    Lipids in Health and Disease, 2007
    Co-Authors: Anthony P James, Karin Slivkoff-clark, John C L Mamo

    Abstract:

    Background A single session of exercise has been reported to reduce fasting and postprandial triacylglycerol concentrations on the subsequent day. It is possible that exercise also reduces chylomicron particle number, which may underlie the observed reduction in postprandial triacylglycerol concentration. In the present study we aimed to determine whether a single session of exercise reduces fasting and postprandial chylomicron particle number on the subsequent day. In a randomised crossover design eight lean and healthy male and female subjects attended two postprandial testing days. On the previous day the subjects either performed 90 minutes of moderate intensity exercise or did not perform any exercise. Fasting blood samples were then collected prior to ingestion of a moderate fat mixed meal (0.44 g fat, 0.94 g carbohydrate, 0.27 g protein/kg body weight), blood was then collected after 1 h, 2 h, 4 h, 6 h, and 8 h. Results The fasting and postprandial Apolipoprotein B48 concentration (marker of chylomicron particle number) was not affected by prior exercise. However exercise reduced fasting triacylglycerol concentration by 16% ( P < 0.05) and there was a trend towards a reduction in the total area under the postprandial triacylglycerol curve (23%; P = 0.053). However when corrected for baseline concentration postprandial triacylglycerol concentration was not affected by prior exercise. Conclusion A single session of exercise of moderate intensity and 90 minutes duration reduces fasting triacylglycerol levels, however fasting and postprandial chylomicron particle number was unaffected. Furthermore it appears that previously observed reductions in postprandial triacylglycerol levels following exercise are only mediated following consumption of high, non-physiologically relevant doses of fat.

  • effect of atorvastatin on Apolipoprotein B48 metabolism and low density lipoprotein receptor activity in normolipidemic patients with coronary artery disease
    Metabolism-clinical and Experimental, 2003
    Co-Authors: Cheryl A Danestewart, Gerald F Watts, Sebely Pal, Dick C Chan, Peter L Thompson, Joseph Hung, John C L Mamo

    Abstract:

    Abstract We aimed to examine postprandial dyslipidemia in normolipidemic patients with coronary artery disease (CAD) and the effects of treatment with an hydroxymethyl glutaryl coenzyme A (HMG-CoA) reductase inhibitor (atorvastatin). Subjects with angiographicaly established CAD were randomized to treatment for 12 weeks with 80 mg/d atorvastatin or placebo and the effects on markers of postprandial lipoproteins and low-density lipoprotein (LDL)-receptor binding determined. LDL-receptor binding was determined in mononuclear cells, as a surrogate for hepatic activity. Fasting levels of cholesterol ( P P 48 ( P = .019), remnant-like particle-cholesterol (RLP-C) ( P = .032), and total postprandial apoB 48 area under the curve (AUC) ( P = .013) significantly decreased with atorvastatin compared with placebo. Atorvastatin also significantly increased LDL-receptor binding activity ( P 48 ( r = .80, P = .01). We report that aberrations in chylomicron metabolism in normolipidemic CAD subjects are correctable with atorvastatin by a mechanism involving increased LDL-receptor activity. This effect may, in part, explain the cardiovascular benefit of statins used in clinical trials of CAD patients with normal lipid levels.

Khosrow Adeli – One of the best experts on this subject based on the ideXlab platform.

  • intestinal scavenger receptor class b type i as a novel regulator of chylomicron production in healthy and diet induced obese states
    American Journal of Physiology-gastrointestinal and Liver Physiology, 2015
    Co-Authors: Marsel Lino, Sarah Farr, Christopher A Baker, Mark T Fuller, Bernardo L Trigatti, Khosrow Adeli

    Abstract:

    The small intestine contributes to diabetic dyslipidemia through the overproduction of Apolipoprotein B48 (apoB48)-containing chylomicron particles. An important regulator of chylomicron generation…

  • glucagon like peptide 2 glp 2 stimulates postprandial chylomicron production and postabsorptive release of intestinal triglyceride storage pools via induction of nitric oxide signaling in male hamsters and mice
    Endocrinology, 2015
    Co-Authors: Joanne Hsieh, Sarah Farr, Karin Trajcevski, Chris Baker, Elizabeth J Lake, Jennifer Taher, Jahangir Iqbal, Mahmood M Hussain, Khosrow Adeli

    Abstract:

    The intestinal overproduction of Apolipoprotein B48 (apoB48)-containing chylomicron particles is a common feature of diabetic dyslipidemia and contributes to cardiovascular risk in insulin resistant states. We previously reported that glucagon-like peptide-2 (GLP-2) is a key endocrine stimulator of enterocyte fat absorption and chylomicron output in the postprandial state. GLP-2’s stimulatory effect on chylomicron production in the postabsorptive state has been confirmed in human studies. The mechanism by which GLP-2 regulates chylomicron production is unclear, because its receptor is not expressed on enterocytes. We provide evidence for a key role of nitric oxide (NO) in mediating the stimulatory effects of GLP-2 during the postprandial and postabsorptive periods. Intestinal chylomicron production was assessed in GLP-2-treated hamsters administered the pan-specific NO synthase (NOS) inhibitor L-NG-nitroarginine methyl ester (L-NAME), and in GLP-2-treated endothelial NOS knockout mice. L-NAME blocked GLP-…

  • cinnamon extract inhibits the postprandial overproduction of Apolipoprotein B48 containing lipoproteins in fructose fed animals
    Journal of Nutritional Biochemistry, 2009
    Co-Authors: Marilyn M Polansky, Yuzo Sato, Khosrow Adeli, Richard A Anderson

    Abstract:

    Abstract We have reported previously that a cinnamon extract (CE), high in type A polyphenols, prevents fructose feeding-induced decreases in insulin sensitivity and suggested that improvements of insulin sensitivity by CE were attributable, in part, to enhanced insulin signaling. In this study, we examined the effects of CE on postprandial Apolipoprotein (apo) B-48 increase in fructose-fed rats, and the secretion of apoB48 in freshly isolated intestinal enterocytes of fructose-fed hamsters. In an olive oil loading study, a water-soluble CE (Cinnulin PF, 50 mg/kg body weight, orally) decreased serum triglyceride (TG) levels and the over production of total- and TG-rich lipoprotein-apoB48. In ex vivo 35 S labeling study, significant decreases were also observed in apoB48 secretion into the media in enterocytes isolated from fructose-fed hamsters. We also investigated the molecular mechanisms of the effects of CE on the expression of genes of the insulin signaling pathway [insulin receptor (IR), IR substrate (IRS)1, IRS2 and Akt1], and lipoprotein metabolism [microsomal TG transfer protein (MTP), sterol regulatory element-binding protein (SREBP1c) in isolated primary enterocytes of fructose-fed hamsters, using quantitative real-time polymerase chain reaction. The CE reversed the expression of the impaired IR, IRS1, IRS2 and Akt1 mRNA levels and inhibited the overexpression of MTP and SREBP1c mRNA levels of enterocytes. Taken together, our data suggest that the postprandial hypertriglycerides and the overproduction of apoB48 can be acutely inhibited by a CE by a mechanism involving improvements of insulin sensitivity of intestinal enterocytes and regulation of MTP and SREBP1c levels. We present both in vivo and ex vivo evidence that a CE improves the postprandial overproduction of intestinal apoB48-containing lipoproteins by ameliorating intestinal insulin resistance and may be beneficial in the control of lipid metabolism.