G Schonfeld

Summary

Affiliation: Washington University School of Medicine
Country: USA

Publications

  1. ncbi request reprint Fatty liver in familial hypobetalipoproteinemia: triglyceride assembly into VLDL particles is affected by the extent of hepatic steatosis
    Gustav Schonfeld
    Department of Internal Medicine, Washington University, St Louis, MO, USA
    J Lipid Res 44:470-8. 2003
  2. ncbi request reprint Familial hypobetalipoproteinemia: a review
    Gustav Schonfeld
    Washington University School of Medicine St Louis, MO 63110, USA
    J Lipid Res 44:878-83. 2003
  3. ncbi request reprint Familial hypobetalipoproteinemia: genetics and metabolism
    G Schonfeld
    Washington University School of Medicine, 660 S Euclid Ave, Campus Box 8046, St Louis, Missouri 63110, USA
    Cell Mol Life Sci 62:1372-8. 2005
  4. pmc Fatty liver and insulin resistance: not always linked
    Gustav Schonfeld
    Washington University School of Medicine, Department of Internal Medicine, St Louis, Missouri 63110, USA
    Trans Am Clin Climatol Assoc 119:217-23; discussion 223-4. 2008
  5. ncbi request reprint Regulation of the apolipoprotein AIV gene expression by estrogen differs in rat and mouse
    R A Srivastava
    Division of Atherosclerosis, Nutrition and Lipid Research, Washington University School of Medicine, Saint Louis, MO 63110
    Eur J Biochem 222:507-14. 1994
  6. ncbi request reprint Regulation of the apolipoprotein B in heterozygous hypobetalipoproteinemic knock-out mice expressing truncated apoB, B81. Low production and enhanced clearance of apoB cause low levels of apoB
    R A Srivastava
    Department of Internal Medicine, Washington University, Saint Louis, MO 63110, USA
    Mol Cell Biochem 202:37-46. 1999
  7. ncbi request reprint A targeted apolipoprotein B-38.9-producing mutation causes fatty livers in mice due to the reduced ability of apolipoprotein B-38.9 to transport triglycerides
    Z Chen
    Division of Atherosclerosis, Nutrition and Lipid Research, Department of Medicine, Washington University School of Medicine, St Louis, Missouri 63110, USA
    J Biol Chem 275:32807-15. 2000
  8. ncbi request reprint Reduced intestinal fat absorptive capacity but enhanced susceptibility to diet-induced fatty liver in mice heterozygous for ApoB38.9 truncation
    Xiaobo Lin
    Department of Medicine, Washington University School of Medicine, St Louis, Missouri 63110, USA
    Am J Physiol Gastrointest Liver Physiol 289:G146-52. 2005
  9. ncbi request reprint Genetic heterogeneity of plasma lipoproteins in the mouse: control of low density lipoprotein particle sizes by genetic factors
    S Jiao
    Department of Internal Medicine, Washington University School of Medicine, St Louis, MO 63110
    J Lipid Res 31:467-77. 1990
  10. ncbi request reprint Estrogen up-regulates apolipoprotein E (ApoE) gene expression by increasing ApoE mRNA in the translating pool via the estrogen receptor alpha-mediated pathway
    R A Srivastava
    Division of Atherosclerosis, Nutrition and Lipid Research, Department of Internal Medicine, Washington University School of Medicine, St Louis, Missouri 63110, USA
    J Biol Chem 272:33360-6. 1997

Research Grants

  1. METABOLISM OF GENETIC VARIANTS OF APOLIPOPROTEIN B
    Gustav Schonfeld; Fiscal Year: 2003
  2. METABOLISM AND GENETICS OF HYPOBETALIPOPROTEINEMIA
    Gustav Schonfeld; Fiscal Year: 2002
  3. METABOLISM AND GENETICS OF HYPOBETALIPOPROTEINEMIA
    Gustav Schonfeld; Fiscal Year: 2007

Detail Information

Publications32

  1. ncbi request reprint Fatty liver in familial hypobetalipoproteinemia: triglyceride assembly into VLDL particles is affected by the extent of hepatic steatosis
    Gustav Schonfeld
    Department of Internal Medicine, Washington University, St Louis, MO, USA
    J Lipid Res 44:470-8. 2003
    ..89 (P = 0.0001) for FHBL subjects and r = 0.69 (P = 0.01) for controls. Thus, apoB truncation-producing mutations result in fatty liver and in altered assembly of VLDL-TG...
  2. ncbi request reprint Familial hypobetalipoproteinemia: a review
    Gustav Schonfeld
    Washington University School of Medicine St Louis, MO 63110, USA
    J Lipid Res 44:878-83. 2003
    ..Hepatic synthesis of fatty acids is reduced in compensation. Informational lacunae remain about genes affecting fat accumulation in liver, and the modulation of liver fat in the presence apoB truncation defects...
  3. ncbi request reprint Familial hypobetalipoproteinemia: genetics and metabolism
    G Schonfeld
    Washington University School of Medicine, 660 S Euclid Ave, Campus Box 8046, St Louis, Missouri 63110, USA
    Cell Mol Life Sci 62:1372-8. 2005
    ..Liver fat in the chromosome 3p21-linked FHBL is normal. Elucidation of the genetic basis of the non-apoB FHBL could uncover attractive targets for lipid-lowering therapy. (See note added in proof.)...
  4. pmc Fatty liver and insulin resistance: not always linked
    Gustav Schonfeld
    Washington University School of Medicine, Department of Internal Medicine, St Louis, Missouri 63110, USA
    Trans Am Clin Climatol Assoc 119:217-23; discussion 223-4. 2008
    ..The genetic/molecular bases for the differences between SWR/J and the other two strains with respect to their glucose metabolic responses to increases in hepatic TG contents remain to be elucidated...
  5. ncbi request reprint Regulation of the apolipoprotein AIV gene expression by estrogen differs in rat and mouse
    R A Srivastava
    Division of Atherosclerosis, Nutrition and Lipid Research, Washington University School of Medicine, Saint Louis, MO 63110
    Eur J Biochem 222:507-14. 1994
    ..In contrast with apoAIV mRNA, estrogen decreased hepatic apoAII mRNA both in rat (threefold) and in mouse (twofold) and parallel changes were observed in transcription rates.(ABSTRACT TRUNCATED AT 400 WORDS)..
  6. ncbi request reprint Regulation of the apolipoprotein B in heterozygous hypobetalipoproteinemic knock-out mice expressing truncated apoB, B81. Low production and enhanced clearance of apoB cause low levels of apoB
    R A Srivastava
    Department of Internal Medicine, Washington University, Saint Louis, MO 63110, USA
    Mol Cell Biochem 202:37-46. 1999
    ..Similar mechanisms appear to contribute to low levels of apoB in hypobetalipoproteinemic humans...
  7. ncbi request reprint A targeted apolipoprotein B-38.9-producing mutation causes fatty livers in mice due to the reduced ability of apolipoprotein B-38.9 to transport triglycerides
    Z Chen
    Division of Atherosclerosis, Nutrition and Lipid Research, Department of Medicine, Washington University School of Medicine, St Louis, Missouri 63110, USA
    J Biol Chem 275:32807-15. 2000
    ..9 may not be responsible for the FHBL phenotypes caused by the apo B-38.9 mutation. Rather, a reduced capacity of apo B-38.9 for triglyceride transport may account for the fatty livers in these mice...
  8. ncbi request reprint Reduced intestinal fat absorptive capacity but enhanced susceptibility to diet-induced fatty liver in mice heterozygous for ApoB38.9 truncation
    Xiaobo Lin
    Department of Medicine, Washington University School of Medicine, St Louis, Missouri 63110, USA
    Am J Physiol Gastrointest Liver Physiol 289:G146-52. 2005
    ..Hepatic TG secretion remained impaired in the apob(+/38.9) on the HF diet. Thus the FHBL mice are more susceptible to diet-induced fatty liver despite relatively reduced intestinal TG absorption capacity on a HF diet...
  9. ncbi request reprint Genetic heterogeneity of plasma lipoproteins in the mouse: control of low density lipoprotein particle sizes by genetic factors
    S Jiao
    Department of Internal Medicine, Washington University School of Medicine, St Louis, MO 63110
    J Lipid Res 31:467-77. 1990
    ..In summary, the genetic control of LDL sizes is more complicated than is the case for HDL; however, the differences in LDL size among these strains of mice may be controlled by a major, as yet unidentified, gene...
  10. ncbi request reprint Estrogen up-regulates apolipoprotein E (ApoE) gene expression by increasing ApoE mRNA in the translating pool via the estrogen receptor alpha-mediated pathway
    R A Srivastava
    Division of Atherosclerosis, Nutrition and Lipid Research, Department of Internal Medicine, Washington University School of Medicine, St Louis, Missouri 63110, USA
    J Biol Chem 272:33360-6. 1997
    ..Thus, estradiol up-regulates apoE gene expression by increasing levels of apoE mRNA in the polysomal translating pool. Furthermore, the increased polysomal recruitment of apoE mRNA is largely mediated by estrogen receptors...
  11. ncbi request reprint In vivo MRS measurement of liver lipid levels in mice
    J R Garbow
    Department of Chemistry and Alvin J Siteman Cancer Center, Washington University, St Louis, MO 63130, USA
    J Lipid Res 45:1364-71. 2004
    ....
  12. ncbi request reprint A targeted apoB38.9 mutation in mice is associated with reduced hepatic cholesterol synthesis and enhanced lipid peroxidation
    Xiaobo Lin
    Department of Medicine, Washington University School of Medicine, St Louis, Missouri, USA
    Am J Physiol Gastrointest Liver Physiol 290:G1170-6. 2006
    ..In contrast, apoB38.9 mice effectively maintain cholesterol homeostasis in the liver, at least in part, by reducing hepatic cholesterol synthesis...
  13. ncbi request reprint Hepatic triglyceride contents are genetically determined in mice: results of a strain survey
    Xiaobo Lin
    660 S Euclid, Campus Box 8046, Department of Medicine, Washington University School of Medicine, St Louis, Missouri 63110, USA
    Am J Physiol Gastrointest Liver Physiol 288:G1179-89. 2005
    ..Additional factor(s) in the development of fatty liver in BALB/cByJ remain to be demonstrated...
  14. ncbi request reprint Heterogeneity of apolipoprotein E epitope expression on human lipoproteins: importance for apolipoprotein E function
    E S Krul
    Department of Medicine, Washington University School of Medicine, St Louis, MO 63110
    J Lipid Res 29:1309-25. 1988
    ....
  15. ncbi request reprint Fenofibrate for the treatment of type IV and V hyperlipoproteinemias: a double-blind, placebo-controlled multicenter US study
    A C Goldberg
    Washington University, St Louis, Missouri
    Clin Ther 11:69-83. 1989
    ..It is concluded that fenofibrate is an effective and safe agent in the treatment of type IV/V hyperlipoproteinemia...
  16. ncbi request reprint Hepatic secretion of apoB-100 is impaired in hypobetalipoproteinemic mice with an apoB-38.9-specifying allele
    Zhouji Chen
    Division of Atherosclerosis, Department of Medicine, Washington University School of Medicine, St Louis, MO 63110, USA
    J Lipid Res 45:155-63. 2004
    ..The greater-than-expected decreases in apoB-100 production rates of FHBL heterozygous humans appear to be attributable to a defect in secretion rather than in the synthesis of apoB-100 from the unaffected apoB allele...
  17. ncbi request reprint Replication of linkage of familial hypobetalipoproteinemia to chromosome 3p in six kindreds
    Rosalind J Neuman
    Washington University School of Medicine, Department of Psychiatry, 660 South Euclid Avenue, Campus Box 8134, St Louis, MO 63110, USA
    J Lipid Res 43:407-15. 2002
    ..1-22. Haplotype analysis identified several meiotic crossover events, allowing us to narrow the critical region from 10 cM to 2.0 cM, between markers D3S2407 and D3S1767...
  18. ncbi request reprint 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
    Zhouji Chen
    Division of Atherosclerosis, Nutrition and Lipid Research, Department of Medicine, Washington University School of Medicine, St Louis, Missouri 63110, USA
    J Biol Chem 277:14135-45. 2002
    ..6-apoB-38.9 peptide segment (aa 1254-1744) plays a critical role, not only in supporting hepatic TG-secretion and in modulating catabolism of apoB-containing lipoproteins, but also in normal mouse embryonic development...
  19. ncbi request reprint Amino terminal 38.9% of apolipoprotein B-100 is sufficient to support cholesterol-rich lipoprotein production and atherosclerosis
    Zhouji Chen
    Department of Medicine, Washington University School of Medicine, Box 8046, 660 South Euclid Avenue, St Louis, MO 63110, USA
    Arterioscler Thromb Vasc Biol 23:668-74. 2003
    ..The atherogenicity of apoB-38.9 was determined in this study by using our apoB-38.9-only (Apob38.9/38.9) mice...
  20. ncbi request reprint Fatty liver in familial hypobetalipoproteinemia: roles of the APOB defects, intra-abdominal adipose tissue, and insulin sensitivity
    Tariq Tanoli
    Departments of Internal Medicine and Radiology, Washington University School of Medicine, St Louis, MO
    J Lipid Res 45:941-7. 2004
    ..001). In summary, because apoB-defective FHBL imparts heightened susceptibility to liver triglyceride accumulation, increasing IPAT and insulin resistance exert greater liver fat-increasing effects in FHBL...
  21. ncbi request reprint Dietary fatty acids and dietary cholesterol differ in their effect on the in vivo regulation of apolipoprotein A-I and A-II gene expression in inbred strains of mice
    R A Srivastava
    Department of Internal Medicine, Washington University School of Medicine, Saint Louis, MO 68110
    Biochim Biophys Acta 1125:251-61. 1992
    ..This study identifies potential loci of regulation and forms the basis for future studies investigating specific genetic and molecular regulatory mechanisms...
  22. pmc Linkage of a gene for familial hypobetalipoproteinemia to chromosome 3p21.1-22
    B Yuan
    Washington University School of Medicine, Washington University, St Louis, MO 63110, USA
    Am J Hum Genet 66:1699-704. 2000
    ..1-22. Weaker linkage to a region near D19S916 was also indicated by Loki and SOLAR. Thus, a heretofore unidentified genetic susceptibility locus for FHBL may reside on chromosome 3...
  23. ncbi request reprint Genetic variants of ApoE account for variability of plasma low-density lipoprotein and apolipoprotein B levels in FHBL
    Pin Yue
    Division of Endocrinology, Metabolism, and Lipid Research, Washington University, School of Medicine, St Louis, MO 63110, USA
    Atherosclerosis 178:107-13. 2005
    ..It accounted for 15-60% of the variability of plasma LDL cholesterol or apoB levels in our FHBL subjects. The physiologic bases for the greater effects of apoE in FHBL remain to be determined...
  24. ncbi request reprint Novel mutations of APOB cause ApoB truncations undetectable in plasma and familial hypobetalipoproteinemia
    Pin Yue
    Division of Atherosclerosis, Nutrition and Lipid Research, Washington University School of Medicine, St Louis, Missouri 63110, USA
    Hum Mutat 20:110-6. 2002
    ..5 readily detectable in plasma. Sequencing of the ApoB gene in families 1, 5, 18, 58, and 59 did not reveal mutations...
  25. ncbi request reprint Hepatic fatty acid synthesis is suppressed in mice with fatty livers due to targeted apolipoprotein B38.9 mutation
    Xiaobo Lin
    Division of Atherosclerosis, Nutrition and Lipid Research, Department of Medicine, Washington University School of Medicine, St Louis, MO 63110, USA
    Arterioscler Thromb Vasc Biol 22:476-82. 2002
    ..The signals mediating suppression of FAS and SREBP-1c levels remain to be identified...
  26. pmc Dissociation between intrahepatic triglyceride content and insulin resistance in familial hypobetalipoproteinemia
    Anastassia Amaro
    Center for Human Nutrition and Atkins Center of Excellence in Obesity Medicine, Washington University School of Medicine, St Louis, Missouri 63110, USA
    Gastroenterology 139:149-53. 2010
    ....
  27. ncbi request reprint Effect of pravastatin on metabolic parameters of apolipoprotein B in patients with mixed hyperlipoproteinemia
    K G Parhofer
    Department of Internal Medicine, Washington University School of Medicine, St Louis
    Clin Investig 71:939-46. 1993
    ..Thus, also in mixed hyperlipoproteinemia 3-hydroxy-3-methylglutaryl coenzyme A reductase inhibitors increase the catabolism of apoB-containing lipoproteins without significantly affecting the production of apoB...
  28. ncbi request reprint Absence of fatty liver in familial hypobetalipoproteinemia linked to chromosome 3p21
    Pin Yue
    Departments of Internal Medicine and Radiology, Washington University School of Medicine, St Louis, MO, USA
    Metabolism 54:682-8. 2005
    ..Lipoprotein profiles and very low density lipoprotein-apoB100 kinetics of 3p21 and apoB-defective groups also differed. Thus, 2 genetic subtypes of FHBL also differ in several phenotypic features...
  29. pmc Truncated apo B-70.5-containing lipoproteins bind to megalin but not the LDL receptor
    Z Chen
    Division of Atherosclerosis, Lipid Research and Nutrition, Department of Medicine, Washington University School of Medicine, St Louis, Missouri 63110, USA
    J Clin Invest 103:1419-30. 1999
    ..Moreover, megalin may play an important role in renal catabolism of apo B truncations, including apo B-70.5...
  30. ncbi request reprint Evidence for a quantitative trait locus affecting low levels of apolipoprotein B and low density lipoprotein on chromosome 10 in Caucasian families
    Richard Sherva
    Washington University School of Medicine, St Louis, MO 63110, USA
    J Lipid Res 48:2632-9. 2007
    ....
  31. ncbi request reprint The c.43_44insCTG variation in PCSK9 is associated with low plasma LDL-cholesterol in a Caucasian population
    Pin Yue
    Department of Internal Medicine, Washington University School of Medicine, St Louis, Missouri, USA
    Hum Mutat 27:460-6. 2006
    ..43_44insCTG variation was approximately 10-15 mg/dL lower than that in normal individuals. We conclude that the c.43_44insCTG variant plays a role in lowering cholesterol in the general population...
  32. doi request reprint The production of 85 kDa N-terminal fragment of apolipoprotein B in mutant HepG2 cells generated by targeted modification of apoB gene occurs by ALLN-inhibitable protease cleavage during translocation
    Neelam Srivastava
    Washington University School of Medicine, St Louis, MO, USA
    Biochem Biophys Res Commun 398:665-70. 2010
    ..These results suggest that the presence of improper folding of apoB during translocation led to the cleavage of both apoB-100 and apoB-82 by ALLN-sensitive protease and generation of 85 kDa N-terminal fragment of apoB...

Research Grants11

  1. METABOLISM OF GENETIC VARIANTS OF APOLIPOPROTEIN B
    Gustav Schonfeld; Fiscal Year: 2003
    ..These broad based studies are expected further to define the relationships between apoB structure and its production, intravascular fate and tissue sites of and cellular catabolism. ..
  2. METABOLISM AND GENETICS OF HYPOBETALIPOPROTEINEMIA
    Gustav Schonfeld; Fiscal Year: 2002
    ..In all these families it has been documented that the affected subjects do not have any evidence of truncated for of apoB. ..
  3. METABOLISM AND GENETICS OF HYPOBETALIPOPROTEINEMIA
    Gustav Schonfeld; Fiscal Year: 2007
    ..abstract_text> ..