H B Brewer

Summary

Affiliation: National Institutes of Health
Country: USA

Publications

  1. ncbi request reprint Regulation of plasma high-density lipoprotein levels by the ABCA1 transporter and the emerging role of high-density lipoprotein in the treatment of cardiovascular disease
    H Bryan Brewer
    Molecular Disease Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD 20892, USA
    Arterioscler Thromb Vasc Biol 24:1755-60. 2004
  2. ncbi request reprint Focus on high-density lipoproteins in reducing cardiovascular risk
    H Bryan Brewer
    Molecular Disease Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD 20892, USA
    Am Heart J 148:S14-8. 2004
  3. ncbi request reprint Hepatic ABCG5/G8 overexpression reduces apoB-lipoproteins and atherosclerosis when cholesterol absorption is inhibited
    Federica Basso
    Molecular Disease Section, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD, USA
    J Lipid Res 48:114-26. 2007
  4. ncbi request reprint Hepatic ABCG5 and ABCG8 overexpression increases hepatobiliary sterol transport but does not alter aortic atherosclerosis in transgenic mice
    Justina E Wu
    Molecular Disease Branch, NHLBI, National Institutes of Health, Bethesda, Maryland 20892, USA
    J Biol Chem 279:22913-25. 2004
  5. ncbi request reprint The farnesoid X-receptor is an essential regulator of cholesterol homeostasis
    Gilles Lambert
    Molecular Disease Branch, NHLBI, National Institutes of Health NIH, Bethesda, Maryland 20892, USA
    J Biol Chem 278:2563-70. 2003
  6. ncbi request reprint Complementary roles of farnesoid X receptor, pregnane X receptor, and constitutive androstane receptor in protection against bile acid toxicity
    Grace L Guo
    Laboratory of Metabolism, NCI, National Institutes of Health, Bethesda, Maryland 20892, USA
    J Biol Chem 278:45062-71. 2003
  7. ncbi request reprint ABCA1 overexpression in the liver of LDLr-KO mice leads to accumulation of pro-atherogenic lipoproteins and enhanced atherosclerosis
    Charles W Joyce
    Molecular Disease Section, NHLBI, National Institutes of Health, Bethesda, MD 20892, USA
    J Biol Chem 281:33053-65. 2006
  8. pmc Conditional disruption of the peroxisome proliferator-activated receptor gamma gene in mice results in lowered expression of ABCA1, ABCG1, and apoE in macrophages and reduced cholesterol efflux
    Taro E Akiyama
    Laboratory of Metabolism, Division of Basic Sciences, National Cancer Institute, National Institutes of Health, Bethesda, Maryland 20892, USA
    Mol Cell Biol 22:2607-19. 2002
  9. ncbi request reprint LCAT modulates atherogenic plasma lipoproteins and the extent of atherosclerosis only in the presence of normal LDL receptors in transgenic rabbits
    M E Brousseau
    Molecular Disease Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD, USA
    Arterioscler Thromb Vasc Biol 20:450-8. 2000
  10. ncbi request reprint The ligand-binding function of hepatic lipase modulates the development of atherosclerosis in transgenic mice
    Herminia Gonzalez-Navarro
    Molecular Disease Branch, NHLBI, National Institutes of Health, Bethesda, Maryland 20892, USA
    J Biol Chem 279:45312-21. 2004

Detail Information

Publications88

  1. ncbi request reprint Regulation of plasma high-density lipoprotein levels by the ABCA1 transporter and the emerging role of high-density lipoprotein in the treatment of cardiovascular disease
    H Bryan Brewer
    Molecular Disease Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD 20892, USA
    Arterioscler Thromb Vasc Biol 24:1755-60. 2004
    ....
  2. ncbi request reprint Focus on high-density lipoproteins in reducing cardiovascular risk
    H Bryan Brewer
    Molecular Disease Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD 20892, USA
    Am Heart J 148:S14-8. 2004
    ....
  3. ncbi request reprint Hepatic ABCG5/G8 overexpression reduces apoB-lipoproteins and atherosclerosis when cholesterol absorption is inhibited
    Federica Basso
    Molecular Disease Section, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD, USA
    J Lipid Res 48:114-26. 2007
    ..These findings identify distinct roles for liver and intestinal ABCG5/G8 in modulating sterol metabolism and atherosclerosis...
  4. ncbi request reprint Hepatic ABCG5 and ABCG8 overexpression increases hepatobiliary sterol transport but does not alter aortic atherosclerosis in transgenic mice
    Justina E Wu
    Molecular Disease Branch, NHLBI, National Institutes of Health, Bethesda, Maryland 20892, USA
    J Biol Chem 279:22913-25. 2004
    ..These findings demonstrate that overexpression of ABCG5/G8 in the liver profoundly alters hepatic but not intestinal sterol transport, identifying distinct roles for liver and intestinal ABCG5/G8 in modulating sterol metabolism...
  5. ncbi request reprint The farnesoid X-receptor is an essential regulator of cholesterol homeostasis
    Gilles Lambert
    Molecular Disease Branch, NHLBI, National Institutes of Health NIH, Bethesda, Maryland 20892, USA
    J Biol Chem 278:2563-70. 2003
    ..These data demonstrate that FXR is a critical regulator of normal cholesterol metabolism and that genetic changes affecting FXR function have the potential to be pro-atherogenic...
  6. ncbi request reprint Complementary roles of farnesoid X receptor, pregnane X receptor, and constitutive androstane receptor in protection against bile acid toxicity
    Grace L Guo
    Laboratory of Metabolism, NCI, National Institutes of Health, Bethesda, Maryland 20892, USA
    J Biol Chem 278:45062-71. 2003
    ..Furthermore, FXR, PXR, and CAR protect against hepatic bile acid toxicity in a complementary manner, suggesting that they serve as redundant but distinct layers of defense to prevent overt hepatic damage by bile acids during cholestasis...
  7. ncbi request reprint ABCA1 overexpression in the liver of LDLr-KO mice leads to accumulation of pro-atherogenic lipoproteins and enhanced atherosclerosis
    Charles W Joyce
    Molecular Disease Section, NHLBI, National Institutes of Health, Bethesda, MD 20892, USA
    J Biol Chem 281:33053-65. 2006
    ....
  8. pmc Conditional disruption of the peroxisome proliferator-activated receptor gamma gene in mice results in lowered expression of ABCA1, ABCG1, and apoE in macrophages and reduced cholesterol efflux
    Taro E Akiyama
    Laboratory of Metabolism, Division of Basic Sciences, National Cancer Institute, National Institutes of Health, Bethesda, Maryland 20892, USA
    Mol Cell Biol 22:2607-19. 2002
    ..Together, these data indicate that PPAR gamma plays a critical role in the regulation of cholesterol homeostasis by controlling the expression of a network of genes that mediate cholesterol efflux from cells and its transport in plasma...
  9. ncbi request reprint LCAT modulates atherogenic plasma lipoproteins and the extent of atherosclerosis only in the presence of normal LDL receptors in transgenic rabbits
    M E Brousseau
    Molecular Disease Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD, USA
    Arterioscler Thromb Vasc Biol 20:450-8. 2000
    ..Moreover, LCAT's antiatherogenic effect requires only a single functional LDLr allele, identifying LCAT as an attractive gene therapy candidate for the majority of dyslipoproteinemic patients...
  10. ncbi request reprint The ligand-binding function of hepatic lipase modulates the development of atherosclerosis in transgenic mice
    Herminia Gonzalez-Navarro
    Molecular Disease Branch, NHLBI, National Institutes of Health, Bethesda, Maryland 20892, USA
    J Biol Chem 279:45312-21. 2004
    ....
  11. ncbi request reprint Two different allelic mutations in the lecithin:cholesterol acyltransferase (LCAT) gene resulting in classic LCAT deficiency: LCAT (tyr83-->stop) and LCAT (tyr156-->asn)
    H G Klein
    Molecular Disease Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD 20892
    J Lipid Res 34:49-58. 1993
    ..In summary, we have identified two unique defects in the LCAT gene that lead to the expression of classic LCAT deficiency in this kindred...
  12. ncbi request reprint Hepatic lipase deficiency decreases the selective uptake of HDL-cholesteryl esters in vivo
    G Lambert
    Molecular Disease Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD 20892, USA
    J Lipid Res 41:667-72. 2000
    ..These data establish for the first time an important role for HL in facilitating the selective uptake of HDL-CE in vivo...
  13. ncbi request reprint Peroxisome proliferator-activated receptor beta/delta regulates very low density lipoprotein production and catabolism in mice on a Western diet
    Taro E Akiyama
    Laboratory of Metabolism, Division of Basic Sciences, National Cancer Institute, National Institutes of Health, Building 37, Bethesda, MD 20892, USA
    J Biol Chem 279:20874-81. 2004
    ....
  14. ncbi request reprint Serum amyloid A promotes ABCA1-dependent and ABCA1-independent lipid efflux from cells
    John A Stonik
    National Institutes of Health Molecular Disease Branch, National Heart, Lung, and Blood Institute, Bethesda, MD, USA
    Biochem Biophys Res Commun 321:936-41. 2004
    ..These results suggest that SAA may play a unique role as an auxiliary lipid acceptor in the removal of lipid from sites of inflammation...
  15. ncbi request reprint The E-box motif in the proximal ABCA1 promoter mediates transcriptional repression of the ABCA1 gene
    Xiao Ping Yang
    Molecular Disease Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD 20892 1666, USA
    J Lipid Res 43:297-306. 2002
    ..These combined studies identify a novel site in the human ABCA1 promoter involved in the regulation of ABCA1 gene expression...
  16. pmc Asymmetry in the lipid affinity of bihelical amphipathic peptides. A structural determinant for the specificity of ABCA1-dependent cholesterol efflux by peptides
    Amar A Sethi
    Lipoprotein Metabolism Section, Pulmonary and Vascular Medicine Branch, NHLBI, National Institutes of Health, Bethesda, Maryland 20892 1508, USA
    J Biol Chem 283:32273-82. 2008
    ..03)). In summary, we describe a novel bihelical peptide with asymmetry in the lipid affinity of its helices and properties similar to apoA-I in terms of specificity for cholesterol efflux by the ABCA1 transporter and low cytotoxicity...
  17. ncbi request reprint Lecithin-cholesterol acyltransferase: role in lipoprotein metabolism, reverse cholesterol transport and atherosclerosis
    S Santamarina-Fojo
    Molecular Disease Branch, National Heart, Lung, and Blood Institute, National Institute of Health, Bethesda, Maryland, USA
    Curr Opin Lipidol 11:267-75. 2000
    ..Analysis of LCAT transgenic animals has established the importance of evaluating HDL function, as well as HDL plasma levels, to predict atherogenic risk...
  18. ncbi request reprint Cellular localization and trafficking of the human ABCA1 transporter
    E B Neufeld
    NHLBI, National Institutes of Health and the NIDDK, National Institutes of Health, Bethesda, Maryland 20892, USA
    J Biol Chem 276:27584-90. 2001
    ..These studies establish a complex intracellular trafficking pathway for human ABCA1 that may play important roles in modulating ABCA1 transporter activity and cellular cholesterol homeostasis...
  19. ncbi request reprint Familial HDL deficiency due to marked hypercatabolism of normal apoA-I
    J Emmerich
    Molecular Disease Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD 20892
    Arterioscler Thromb 13:1299-306. 1993
    ..This study establishes that a form of severe hypoalphalipoproteinemia distinct from Tangier disease can be caused by marked hypercatabolism of a normal A-I apolipoprotein...
  20. ncbi request reprint Rising to the challenge of the new NCEP ATP III guidelines: exceeding current therapeutic limitations
    H Bryan Brewer
    Molecular Disease Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland, USA
    Am J Manag Care 8:S23-8; discussion S45-7. 2002
    ..These mechanisms and others also suggest new targets for therapeutic intervention and the development of new drugs that will correct lipid and lipoprotein abnormalities through a number of different metabolic pathways...
  21. pmc Selective delipidation of plasma HDL enhances reverse cholesterol transport in vivo
    Frank M Sacks
    Harvard School of Public Health and Harvard Medical School, Boston, MA, USA, USA
    J Lipid Res 50:894-907. 2009
    ..Treatment with delipidated plasma tended to reduce diet-induced aortic atherosclerosis in monkeys measured by intravascular ultrasound. These findings link the conversion of small to large HDL, in vivo, to improvement in atherosclerosis...
  22. ncbi request reprint Low-density lipoprotein receptor and apolipoprotein A-I and B expression in human enterocytes
    Dmitri Sviridov
    Molecular Disease Branch, National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, MD, USA
    Digestion 67:67-70. 2003
    ..Immunoprecipitation of [(35)S]methionine pulse-labeled intracellular proteins from these cell types demonstrated that human enterocytes synthesize more apoA-I and apoB, while HepG2 cells synthesize a slightly higher amount of LDL-R...
  23. ncbi request reprint Apolipoprotein specificity for lipid efflux by the human ABCAI transporter
    A T Remaley
    National Heart, Lung and Blood Institute, Bethesda, Maryland 20982, USA
    Biochem Biophys Res Commun 280:818-23. 2001
    ..In summary, ABCAI-mediated cellular binding of apolipoproteins and lipid efflux is not specific for only apoA-I but can also occur with other apolipoproteins that contain multiple amphipathic helical domains...
  24. pmc Complete genomic sequence of the human ABCA1 gene: analysis of the human and mouse ATP-binding cassette A promoter
    S Santamarina-Fojo
    National Heart, Lung, and Blood Institute, and Clinical Center, Clinical Pathology Department, National Institutes of Health, Bethesda, MD 20892, USA
    Proc Natl Acad Sci U S A 97:7987-92. 2000
    ..These combined findings provide insights into ABCA1-mediated regulation of cellular cholesterol metabolism and will facilitate the identification of new pharmacologic agents for the treatment of atherosclerosis in humans...
  25. ncbi request reprint Analysis of glomerulosclerosis and atherosclerosis in lecithin cholesterol acyltransferase-deficient mice
    G Lambert
    Molecular Disease Branch, NHLBI, National Institutes of Health, Bethesda, Maryland 20892, USA
    J Biol Chem 276:15090-8. 2001
    ....
  26. ncbi request reprint Tissue-specific expression of apolipoprotein A-I (ApoA-I) is regulated by the 5'-flanking region of the human ApoA-I gene
    K Higuchi
    Molecular Disease Branch, National Heart, Lung, and Blood Institute, Bethesda, Maryland 20892
    J Biol Chem 263:18530-6. 1988
    ....
  27. ncbi request reprint Targeted disruption of the mouse lecithin:cholesterol acyltransferase (LCAT) gene. Generation of a new animal model for human LCAT deficiency
    N Sakai
    Molecular Disease Branch, NHLBI, National Institutes of Health, Bethesda, Maryland 20892 1666, USA
    J Biol Chem 272:7506-10. 1997
    ....
  28. ncbi request reprint The ABCA1 transporter modulates late endocytic trafficking: insights from the correction of the genetic defect in Tangier disease
    Edward B Neufeld
    Molecular Disease Branch, NHLBI, NHLBI Light Microscopy Core Facility, and Laboratory for Cellular Biology and Biochemistry, NIDDK, National Institutes of Health, Bethesda, Maryland 20892, USA
    J Biol Chem 279:15571-8. 2004
    ..The combined results of this study suggest that ABCA1 converts pools of late endocytic lipids that retain NPC1 to pools that can associate with endocytosed apoA-I, and be released from the cell as nascent high density lipoprotein...
  29. ncbi request reprint Comparative genome analysis of potential regulatory elements in the ABCG5-ABCG8 gene cluster
    Alan T Remaley
    National Heart, Lung and Blood Institute, National Institutes of Health, Bldg 10 2C 433, 10 Center Drive, Bethesda, MD 20892, USA
    Biochem Biophys Res Commun 295:276-82. 2002
    ..In summary, several potential regulatory elements were found for the ABCG5 and ABCG8 genes, and the intergenic region was found to act as a bidirectional promoter...
  30. ncbi request reprint The ABCA1 transporter functions on the basolateral surface of hepatocytes
    Edward B Neufeld
    Molecular Disease Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, 10 7N115, 10 Center Drive, 20892, Bethesda, MD 20892, USA
    Biochem Biophys Res Commun 297:974-9. 2002
    ..These findings suggest an important role for hepatocyte basolateral membrane ABCA1 in the regulation of the levels of intracellular hepatic cholesterol, as well as plasma HDL...
  31. ncbi request reprint Developmental regulation of apolipoprotein B mRNA editing is an autonomous function of small intestine involving homeobox gene Cdx1
    Amy P Patterson
    NHLBI, National Institutes of Health, Bethesda, Maryland 20892, USA
    J Biol Chem 278:7600-6. 2003
    ..Taken together, these data suggest that the developmental regulation of apoB mRNA editing is an autonomous cytodifferentiation function of small intestine for which homeobox gene Cdx1 may play an important role...
  32. ncbi request reprint Synthetic amphipathic helical peptides promote lipid efflux from cells by an ABCA1-dependent and an ABCA1-independent pathway
    Alan T Remaley
    National Institutes of Health Molecular Disease Branch, National Heart, Lung, and Blood Institute, Bethesda, MD 20892, USA
    J Lipid Res 44:828-36. 2003
    ..In addition, unlike apoA-I, synthetic peptides can also efflux lipid by a passive, energy-independent pathway that does not involve ABCA1 but does depend upon their lipid affinity...
  33. ncbi request reprint Role of the hepatic ABCA1 transporter in modulating intrahepatic cholesterol and plasma HDL cholesterol concentrations
    Federica Basso
    Molecular Disease Branch, NHLBI, National Institutes of Health, Bethesda, MD 20862, USA
    J Lipid Res 44:296-302. 2003
    ..These combined results demonstrate that ABCA1 plays a key role in hepatic cholesterol efflux, inducing pathways that modulate cholesterol homeostasis in the liver, and establish the liver as a major source of plasma HDL-C...
  34. ncbi request reprint The orphan nuclear receptor LRH-1 activates the ABCG5/ABCG8 intergenic promoter
    Lita A Freeman
    Molecular Disease Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD, USA
    J Lipid Res 45:1197-206. 2004
    ....
  35. ncbi request reprint Study of ABCA1 function in transgenic mice
    Charles Joyce
    Molecular Disease Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD 20892, USA
    Arterioscler Thromb Vasc Biol 23:965-71. 2003
    ..The present review summarizes the most recent studies and discusses insights provided by these transgenic mouse models...
  36. ncbi request reprint New insights into the role of the adenosine triphosphate-binding cassette transporters in high-density lipoprotein metabolism and reverse cholesterol transport
    H Bryan Brewer
    Molecular Disease Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland 20892, USA
    Am J Cardiol 91:3E-11E. 2003
    ..The ABC transporters currently represent excellent targets for the development of new drugs for the treatment of patients with increased risk of premature cardiovascular disease...
  37. ncbi request reprint New features of the National Cholesterol Education Program Adult Treatment Panel III lipid-lowering guidelines
    H Bryan Brewer
    Molecular Disease Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland 20814, USA
    Clin Cardiol 26:III19-24. 2003
    ..These new features of the NCEP ATP III guidelines should improve identification and treatment of patients with dyslipidemias associated with CHD risk...
  38. ncbi request reprint Increasing HDL Cholesterol Levels
    H Bryan Brewer
    National Heart, Lung, and Blood Institute, Bethesda, MD, USA
    N Engl J Med 350:1491-4. 2004
  39. ncbi request reprint Benefit-risk assessment of Rosuvastatin 10 to 40 milligrams
    H Bryan Brewer
    Molecular Disease Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland 20894, USA
    Am J Cardiol 92:23K-29K. 2003
    ..2% of patients receiving rosuvastatin and those receiving atorvastatin, simvastatin, and pravastatin. Compared with other widely used statins, the benefit-risk profile of rosuvastatin 10 to 40 mg appears to be very favorable...
  40. ncbi request reprint Hepatic CCAAT/enhancer binding protein alpha mediates induction of lipogenesis and regulation of glucose homeostasis in leptin-deficient mice
    Kimihiko Matsusue
    Laboratory of Metabolism, National Cancer Institute, National Institutes of Health, Bethesda, Maryland 20892, USA
    Mol Endocrinol 18:2751-64. 2004
    ..Taken together, these results indicate that hepatic C/EBP alpha plays a critical role in the acceleration of lipogenesis in ob/ob mice and in glucose homeostasis by the indirect regulation of insulin secretion...
  41. ncbi request reprint The human ABCG1 gene: identification of LXR response elements that modulate expression in macrophages and liver
    Steven L Sabol
    Molecular Disease Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD 20892, USA
    J Lipid Res 46:2151-67. 2005
    ..These studies clarify the mechanism of transcriptional upregulation of the ABCG1 gene by oxysterols in macrophages and liver, two key tissues where ABCG1 expression may affect cholesterol balance and atherogenesis...
  42. ncbi request reprint Clinical significance of high-density lipoproteins and the development of atherosclerosis: focus on the role of the adenosine triphosphate-binding cassette protein A1 transporter
    H Bryan Brewer
    Molecular Disease Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland 20894, USA
    Am J Cardiol 92:10K-16K. 2003
    ..Ongoing investigation of mechanisms by which HDL acts to reduce the risk of atherosclerosis will provide several new targets for the development of drugs to decrease the risk of atherosclerosis...
  43. ncbi request reprint Lipolytic and ligand-binding functions of hepatic lipase protect against atherosclerosis in LDL receptor-deficient mice
    Lita Freeman
    Molecular Disease Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD 20892, USA
    J Lipid Res 48:104-13. 2007
    ..These changes, evident even in the presence of apoE, establish an antiatherogenic role of the ligand-binding function of HL in LDLr-deficient mice...
  44. pmc The ATP binding cassette transporter A1 (ABCA1) modulates the development of aortic atherosclerosis in C57BL/6 and apoE-knockout mice
    Charles W Joyce
    Molecular Disease Branch, National Heart, Lung, and Blood Institute, Bethesda, MD 20892, USA
    Proc Natl Acad Sci U S A 99:407-12. 2002
    ....
  45. ncbi request reprint Abetalipoproteinemia. New insights into lipoprotein assembly and vitamin E metabolism from a rare genetic disease
    D J Rader
    Molecular Disease Branch, National Heart, Lung, and Blood Institute, Bethesda, MD 20892
    JAMA 270:865-9. 1993
    ..The systematic investigation of this rare genetic disease has provided insights that have substantially enhanced our understanding of human physiology...
  46. ncbi request reprint Regulation and intracellular trafficking of the ABCA1 transporter
    S Santamarina-Fojo
    Molecular Disease Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD 20892, USA
    J Lipid Res 42:1339-45. 2001
    ..Given the key role that ABCA1 plays in cholesterol homeostasis, it is likely that there are multiple mechanisms for controlling the overall transporter activity of ABCA1...
  47. pmc In vivo metabolism of proapolipoprotein A-I in Tangier disease
    D Bojanovski
    Molecular Disease Branch, National Heart, Lung, and Blood Institute, Bethesda, Maryland 20892
    J Clin Invest 80:1742-7. 1987
    ..Therefore, the relative increase in proapoA-I in Tangier disease is due to a marked decrease in mature apoA-I resulting from rapid catabolism of both pro- and mature apoA-I and not to defective conversion of proapoA-I to mature apoA-I...
  48. pmc Two different allelic mutations in the lecithin-cholesterol acyltransferase gene associated with the fish eye syndrome. Lecithin-cholesterol acyltransferase (Thr123----Ile) and lecithin-cholesterol acyltransferase (Thr347----Met)
    H G Klein
    Molecular Disease Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland 20892
    J Clin Invest 89:499-506. 1992
    ..Our data indicate that the regions adjacent to Thr123 and Thr347 of LCAT may play an important role in HDL cholesterol esterification, suggesting that these regions may contain a portion of the LCAT binding domain(s) for HDL...
  49. ncbi request reprint In vivo metabolism of apolipoproteins A-I and E in patients with abetalipoproteinemia: implications for the roles of apolipoproteins B and E in HDL metabolism
    K Ikewaki
    Molecular Disease Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD 20892
    J Lipid Res 35:1809-19. 1994
    ..Among the three different forms of apoE, the apoE monomer was catabolized at the fastest rate, the apoE homodimer at an intermediate rate, and the apoE-A-II heterodimer had the slowest rate of catabolism.(ABSTRACT TRUNCATED AT 400 WORDS)..
  50. pmc Human ATP-binding cassette transporter 1 (ABC1): genomic organization and identification of the genetic defect in the original Tangier disease kindred
    A T Remaley
    National Institutes of Health, National Heart, Lung and Blood Institute, Bethesda, MD 20892, USA
    Proc Natl Acad Sci U S A 96:12685-90. 1999
    ..These results will be useful in the future characterization of the structure and function of the ABC1 gene and the analysis of additional ABC1 mutations in patients with Tangier disease...
  51. pmc Fish eye syndrome: a molecular defect in the lecithin-cholesterol acyltransferase (LCAT) gene associated with normal alpha-LCAT-specific activity. Implications for classification and prognosis
    H G Klein
    Molecular Disease Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland 20892
    J Clin Invest 92:479-85. 1993
    ....
  52. ncbi request reprint Abnormal in vivo metabolism of apoB-containing lipoproteins in human apoE deficiency
    Katsunori Ikewaki
    Molecular Disease Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD 20892, USA
    J Lipid Res 45:1302-11. 2004
    ....
  53. ncbi request reprint In vivo metabolism of apolipoprotein E within the HDL subpopulations LpE, LpE:A-I, LpE:A-II and LpE:A-I:A-II
    Minna L Hannuksela
    Molecular Disease Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD, USA
    Atherosclerosis 165:205-20. 2002
    ..These differences were related to the lipid and apolipoprotein composition of the HDL subspecies, and, in control subjects, to the transfer of apoE from HDL subspecies to apoB-containing lipoproteins as well...
  54. ncbi request reprint Human lecithin:cholesterol acyltransferase deficiency: in vivo kinetics of low-density lipoprotein and lipoprotein-X
    Masato Nishiwaki
    Molecular Disease Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD, USA
    Arterioscler Thromb Vasc Biol 26:1370-5. 2006
    ..In addition, the mechanism(s) for LpX accumulation is not known. The aim of the present study is to elucidate the mechanism(s) responsible for the low LDL and determine the plasma kinetics of LpX in LCAT-def...
  55. pmc ABCA1 overexpression leads to hyperalphalipoproteinemia and increased biliary cholesterol excretion in transgenic mice
    B L Vaisman
    Molecular Disease Branch, National Heart, Lung, and Blood Institute, Bethesda, Maryland 20892 1666, USA
    J Clin Invest 108:303-9. 2001
    ..Similar metabolic changes may modify atherogenic risk in humans...
  56. ncbi request reprint MR virtual angioscopy of thoracic aortic atherosclerosis in homozygous familial hypercholesterolemia
    R M Summers
    Diagnostic Radiology Department, Warren Grant Magnuson Clinical Center, National Institutes of Health, Bethesda, MD 20892 1182, USA
    J Comput Assist Tomogr 25:371-7. 2001
    ..Thoracic aortic atherosclerosis in patients with HFH was assessed with contrast-enhanced MR angiograms using exoscopic and endoscopic virtual angioscopy reconstructions and maximum intensity projections (MIPs)...
  57. ncbi request reprint Erdheim-Chester disease: a rare multisystem histiocytic disorder associated with interstitial lung disease
    R D Shamburek
    Molecular Disease Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland 20892 1666, USA
    Am J Med Sci 321:66-75. 2001
    ..In general, the clinical course of patients with this disease varies, and the prognosis can be poor despite treatment. Clinical trials for treatment of ECD have not been conducted and treatment is based on anecdotal experience...
  58. pmc Lipoprotein lipaseBethesda: a single amino acid substitution (Ala-176----Thr) leads to abnormal heparin binding and loss of enzymic activity
    O U Beg
    Molecular Disease Branch, National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, MD 20892
    Proc Natl Acad Sci U S A 87:3474-8. 1990
    ..We propose that an amino acid substitution in this critical region of LPLBethesda results in the synthesis of a nonfunctional enzyme that leads to the chylomicronemia syndrome expressed in this proband...
  59. ncbi request reprint Human lipoprotein lipase. Analysis of the catalytic triad by site-directed mutagenesis of Ser-132, Asp-156, and His-241
    J Emmerich
    Molecular Disease Branch, National Heart Lung and Blood Institute, Bethesda, Maryland 20892
    J Biol Chem 267:4161-5. 1992
    ..These combined results strongly support the conclusion that Ser-132, Asp-156, and His-241 form the catalytic triad of LPL and are essential for LPL hydrolytic activity...
  60. ncbi request reprint A deletion mutation in the ApoC-II gene (ApoC-II Nijmegen) of a patient with a deficiency of apolipoprotein C-II
    S S Fojo
    Molecular Disease Branch, National Heart, Lung, and Blood Institute, Nijmegen, The Netherlands
    J Biol Chem 263:17913-6. 1988
    ....
  61. ncbi request reprint Identification of two separate allelic mutations in the lipoprotein lipase gene of a patient with the familial hyperchylomicronemia syndrome
    H L Dichek
    Molecular Disease Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland 20892
    J Biol Chem 266:473-7. 1991
    ....
  62. pmc Dominant expression of type III hyperlipoproteinemia. Pathophysiological insights derived from the structural and kinetic characteristics of ApoE-1 (Lys146-->Glu)
    W A Mann
    Molecular Disease Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland 20892, USA
    J Clin Invest 96:1100-7. 1995
    ..These abnormal in vitro binding characteristics and the altered in vivo metabolism of apoE-1 (Lys146-->Glu) are proposed to result in the functional dominance of this mutation in the affected kindred...
  63. pmc Expression of apolipoprotein B mRNAs encoding higher- and lower-molecular weight isoproteins in rat liver and intestine
    G E Tennyson
    Molecular Disease Branch, National Heart, Lung, and Blood Institute, Bethesda, MD 20892
    Proc Natl Acad Sci U S A 86:500-4. 1989
    ....
  64. pmc Donor splice site mutation in the apolipoprotein (Apo) C-II gene (Apo C-IIHamburg) of a patient with Apo C-II deficiency
    S S Fojo
    Molecular Disease Branch, National Heart, Lung and Blood Institute, Bethesda, Maryland 20892
    J Clin Invest 82:1489-94. 1988
    ..We propose that this donor splice site mutation is the primary genetic defect that leads to defective splicing and ultimately to an apo C-II deficiency in this kindred...
  65. ncbi request reprint Phosphorylation and modulation of the enzymic activity of native and protease-cleaved purified hepatic 3-hydroxy-3-methylglutaryl-coenzyme A reductase by a calcium/calmodulin-dependent protein kinase
    Z H Beg
    Molecular Disease Branch, National Heart, Lung, and Blood Institute, Bethesda, Maryland 20892
    J Biol Chem 262:13228-40. 1987
    ....
  66. ncbi request reprint Characterization of high density lipoprotein-bound and soluble RT6 released following administration of anti-RT6.1 monoclonal antibody
    E Lesma
    Pulmonary Critical Care Medicine Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD 20892, USA
    J Immunol 161:1212-9. 1998
    ..Since GPI-linked proteins in HDL can transfer to cells in a functionally active form, the presence of RT6 in HDL is consistent with it having a role in signaling in nonlymphoid cells...
  67. ncbi request reprint Three genetic variants of human plasma apolipoprotein A-IV. apoA-IV-1(Thr347----Ser), apoA-IV-0(Lys167----Glu,Gln360----His), and apoA-IV-3(Glu165----Lys)
    P Lohse
    Molecular Disease Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland 20892
    J Biol Chem 266:13513-8. 1991
    ..These results indicate that one predominant allele codes for the isoproteins apoA-IV-2 and apoA-IV-0 and that at least two major allelic variants for the isoproteins apoA-IV-1 and apoA-IV-3 are present in the population analyzed...
  68. ncbi request reprint Apolipoprotein E-4Philadelphia (Glu13----Lys,Arg145----Cys). Homozygosity for two rare point mutations in the apolipoprotein E gene combined with severe type III hyperlipoproteinemia
    P Lohse
    Molecular Disease Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland 20892
    J Biol Chem 266:10479-84. 1991
    ..Using these restriction fragment length polymorphisms as well as DNA sequence analysis we have demonstrated that the patient is homozygous for both point mutations in the apoE gene...
  69. ncbi request reprint Human plasma apolipoproteins A-IV-0 and A-IV-3. Molecular basis for two rare variants of apolipoprotein A-IV-1
    P Lohse
    Molecular Disease Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland 20892
    J Biol Chem 265:12734-9. 1990
    ..08). Comparison with the mouse and rat A-IV apolipoproteins revealed that this residue, located at position 4 of the 10th/11th amphiphilic alpha-helical repeat, is also highly conserved in evolution...
  70. pmc Human apolipoprotein B (apoB) mRNA: identification of two distinct apoB mRNAs, an mRNA with the apoB-100 sequence and an apoB mRNA containing a premature in-frame translational stop codon, in both liver and intestine
    K Higuchi
    Molecular Disease Branch, National Heart, Lung, and Blood Institute, Bethesda, MD 20892
    Proc Natl Acad Sci U S A 85:1772-6. 1988
    ..The premature in-frame stop codon is not tissue specific and is present in both human liver and intestine...
  71. ncbi request reprint Genetic polymorphism of human plasma apolipoprotein A-IV is due to nucleotide substitutions in the apolipoprotein A-IV gene
    P Lohse
    Molecular Disease Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland 20892
    J Biol Chem 265:10061-4. 1990
    ....
  72. ncbi request reprint Identification of a novel in-frame translational stop codon in human intestine apoB mRNA
    A V Hospattankar
    Molecular Disease Branch, National Heart, Lung, and Blood Institute, Bethesda, MD 20892
    Biochem Biophys Res Commun 148:279-85. 1987
    ..The new stop codon in the human intestinal apoB mRNA provides a potential mechanism for the biosynthesis of intestinal apoB-48...
  73. ncbi request reprint Familial apolipoprotein E deficiency and type III hyperlipoproteinemia due to a premature stop codon in the apolipoprotein E gene
    P Lohse
    Molecular Disease Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD 20892
    J Lipid Res 33:1583-90. 1992
    ..The clinical presentation also suggests a dispensable role of apoE in the nervous system and in immunoregulation...
  74. pmc A nonsense mutation in the apolipoprotein C-IIPadova gene in a patient with apolipoprotein C-II deficiency
    S S Fojo
    Molecular Disease Branch, National Heart, Lung, and Blood Institute, Bethesda, Maryland 20892
    J Clin Invest 84:1215-9. 1989
    ..We propose that the C to A substitution in the apo C-IIPadova gene is the primary genetic defect that leads to premature termination and the synthesis of a truncated 36 amino acid apo C-II that is unable to activate lipoprotein lipase...
  75. ncbi request reprint A new dawn in the treatment of dyslipidemia: cardiovascular risk reduction through emerging science and proven clinical results. Introduction
    H Bryan Brewer
    Molecular Disease Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD, USA
    Am Heart J 148:S1-2. 2004
  76. ncbi request reprint HDL metabolism and the role of HDL in the treatment of high-risk patients with cardiovascular disease
    H Bryan Brewer
    Department of Lipoprotein and Atherosclerosis Research, Cardiovascular Research Institute, MedStar Research Institute, Washington Hospital Center, Washington, DC 20010, USA
    Curr Cardiol Rep 9:486-92. 2007
    ..Definitive clinical trials will now be required to establish the safety and efficacy of increasing HDL in the treatment of patients with cardiovascular disease...
  77. ncbi request reprint Wild-type PCSK9 inhibits LDL clearance but does not affect apoB-containing lipoprotein production in mouse and cultured cells
    Florent Lalanne
    Institut National de la Santé et de la Recherche Médicale U539, Centre Hospitalier Universitaire, Hotel Dieu, Nantes, France
    J Lipid Res 46:1312-9. 2005
    ..Finally, we show that unlike PCSK9 overexpression in mice, the S127R mutation in patients led to increased VLDL apoB levels, suggesting a potential gain of function for S127R-PCSK9 in humans...
  78. ncbi request reprint Implications of recent clinical trials for the National Cholesterol Education Program Adult Treatment Panel III Guidelines
    Scott M Grundy
    J Am Coll Cardiol 44:720-32. 2004
    ..Finally, for people in lower-risk categories, recent clinical trials do not modify the goals and cutpoints of therapy...
  79. ncbi request reprint Effect of ACAT inhibition on the progression of coronary atherosclerosis
    Steven E Nissen
    Department of Cardiovascular Medicine, Cleveland Clinic Foundation, Cleveland, OH 44195, USA
    N Engl J Med 354:1253-63. 2006
    ..The enzyme acyl-coenzyme A:cholesterol acyltransferase (ACAT) esterifies cholesterol in a variety of tissues. In some animal models, ACAT inhibitors have antiatherosclerotic effects...
  80. ncbi request reprint Implications of recent clinical trials for the National Cholesterol Education Program Adult Treatment Panel III guidelines
    Scott M Grundy
    Arterioscler Thromb Vasc Biol 24:e149-61. 2004
    ..Finally, for people in lower-risk categories, recent clinical trials do not modify the goals and cutpoints of therapy...
  81. ncbi request reprint A summary of implications of recent clinical trials for the National Cholesterol Education Program Adult Treatment Panel III guidelines
    Scott M Grundy
    Arterioscler Thromb Vasc Biol 24:1329-30. 2004
  82. ncbi request reprint High-density lipoproteins: a new potential therapeutic target for the prevention of cardiovascular disease
    H Bryan Brewer
    Arterioscler Thromb Vasc Biol 24:387-91. 2004
  83. ncbi request reprint Implications of recent clinical trials for the National Cholesterol Education Program Adult Treatment Panel III guidelines
    Scott M Grundy
    Circulation 110:227-39. 2004
    ..Finally, for people in lower-risk categories, recent clinical trials do not modify the goals and cutpoints of therapy...
  84. ncbi request reprint Definition of metabolic syndrome: report of the National Heart, Lung, and Blood Institute/American Heart Association conference on scientific issues related to definition
    Scott M Grundy
    Arterioscler Thromb Vasc Biol 24:e13-8. 2004
  85. ncbi request reprint Cholesteryl ester transfer protein: a novel target for raising HDL and inhibiting atherosclerosis
    Philip J Barter
    Hanson Institute and the Department of Cardiology, Royal Adelaide Hospital, Adelaide, Australia
    Arterioscler Thromb Vasc Biol 23:160-7. 2003
    ..Thus, it seems important and timely to test the hypothesis in randomized trials of humans that pharmacological inhibition of CETP retards the development of atherosclerosis...
  86. ncbi request reprint Impact of low high-density lipoproteins on in-hospital events and one-year clinical outcomes in patients with non-ST-elevation myocardial infarction acute coronary syndrome treated with drug-eluting stent implantation
    Roswitha M Wolfram
    The Division of Cardiology, Washington Hospital Center, Washington, DC, USA
    Am J Cardiol 98:711-7. 2006
    ..15 to 10.0) at 1 year. In conclusion, regardless of baseline low-density lipoprotein cholesterol levels and statin therapy, additional strategies to increase HDL cholesterol should be evaluated in patients with acute coronary syndrome...
  87. ncbi request reprint Comparative in vivo metabolism of apolipoproteins E2 and E4 in heterozygous apoE2/4 subjects
    Katsunori Ikewaki
    Department of Cardiology, Jikei University School of Medicine, Tokyo, Japan
    J Lab Clin Med 140:369-74. 2002
    ..In conclusion, E4 is catabolized three times faster than apoE2 in heterozygous E2/4 subjects, indicating that these two apoE isoproteins have distinct metabolic pathways...
  88. ncbi request reprint Definition of metabolic syndrome: Report of the National Heart, Lung, and Blood Institute/American Heart Association conference on scientific issues related to definition
    Scott M Grundy
    Circulation 109:433-8. 2004