Stephen G Young

Summary

Affiliation: University of California
Country: USA

Publications

  1. Birrane G, Beigneux A, Dwyer B, Strack Logue B, Kristensen K, Francone O, et al. Structure of the lipoprotein lipase-GPIHBP1 complex that mediates plasma triglyceride hydrolysis. Proc Natl Acad Sci U S A. 2019;116:1723-1732 pubmed publisher
    ..The LPL-GPIHBP1 structure provides insights into mutations causing chylomicronemia. ..
  2. Kristensen K, Midtgaard S, Mysling S, Kovrov O, Hansen L, Skar Gislinge N, et al. A disordered acidic domain in GPIHBP1 harboring a sulfated tyrosine regulates lipoprotein lipase. Proc Natl Acad Sci U S A. 2018;115:E6020-E6029 pubmed publisher
  3. He C, Hu X, Jung R, Weston T, Sandoval N, Tontonoz P, et al. High-resolution imaging and quantification of plasma membrane cholesterol by NanoSIMS. Proc Natl Acad Sci U S A. 2017;114:2000-2005 pubmed publisher
    ..These images demonstrate that accessible cholesterol, as judged by PFO* or ALO-D4 binding, is not evenly distributed over the entire plasma membrane but instead is highly enriched on microvilli. ..
  4. Yang S, Procaccia S, Jung H, Nobumori C, Tatar A, Tu Y, et al. Mice that express farnesylated versions of prelamin A in neurons develop achalasia. Hum Mol Genet. 2015;24:2826-40 pubmed publisher
    ..Our findings indicate that progerin and full-length farnesyl-prelamin A are toxic to neurons of the enteric nervous system. ..
  5. Lee J, Tu Y, Tatar A, Wu D, Nobumori C, Jung H, et al. Reciprocal knock-in mice to investigate the functional redundancy of lamin B1 and lamin B2. Mol Biol Cell. 2014;25:1666-75 pubmed publisher
    ..We conclude that lamins B1 and B2 have unique roles in the developing brain and that increased production of one B-type lamin does not fully complement loss of the other. ..
  6. Allan C, Heizer P, Tu Y, Sandoval N, Jung R, Morales J, et al. An upstream enhancer regulates Gpihbp1 expression in a tissue-specific manner. J Lipid Res. 2018;: pubmed publisher
    ..The enhancer element that we identified represents the first insight into DNA sequences controlling Gpihbp1 expression. ..
  7. Eguchi J, Miyashita K, Fukamachi I, Nakajima K, Murakami M, Kawahara Y, et al. GPIHBP1 autoantibody syndrome during interferon β1a treatment. J Clin Lipidol. 2019;13:62-69 pubmed publisher
    ..The appearance of GPIHBP1 autoantibodies during IFN β1a therapy caused chylomicronemia. The GPIHBP1 autoantibodies disappeared when the IFN β1a therapy was stopped, and the plasma triglyceride levels fell within the normal range. ..
  8. He C, Hu X, Weston T, Jung R, Heizer P, Tu Y, et al. NanoSIMS imaging reveals unexpected heterogeneity in nutrient uptake by brown adipocytes. Biochem Biophys Res Commun. 2018;504:899-902 pubmed publisher
    ..The uptake of nutrients by adjacent brown adipocytes within a single depot is variable, suggesting that there is heterogeneity in the metabolic properties of brown adipocytes. ..
  9. He C, Weston T, Jung R, Heizer P, Larsson M, Hu X, et al. NanoSIMS Analysis of Intravascular Lipolysis and Lipid Movement across Capillaries and into Cardiomyocytes. Cell Metab. 2018;27:1055-1066.e3 pubmed publisher
    ..Remarkably, a deficiency of the putative fatty acid transport protein CD36, which is expressed highly in capillary endothelial cells, did not impede entry of TRL-derived lipids into cardiomyocytes. ..

More Information

Publications21

  1. Turlo K, Leung C, Seo J, Goulbourne C, Adeyo O, Gin P, et al. Equivalent binding of wild-type lipoprotein lipase (LPL) and S447X-LPL to GPIHBP1, the endothelial cell LPL transporter. Biochim Biophys Acta. 2014;1841:963-9 pubmed publisher
    ..We conclude that increased binding of S447X-LPL to GPIHBP1 is unlikely to be the explanation for more efficient lipolysis and lower plasma triglyceride levels in S447X carriers. ..
  2. Young S, Davies B, Voss C, Gin P, Weinstein M, Tontonoz P, et al. GPIHBP1, an endothelial cell transporter for lipoprotein lipase. J Lipid Res. 2011;52:1869-84 pubmed publisher
    ..We also discuss the human genetics of LPL transport, focusing on cases of chylomicronemia caused by GPIHBP1 mutations that abolish GPIHBP1's ability to bind LPL, and LPL mutations that prevent LPL binding to GPIHBP1. ..
  3. Young S, Davies B, Fong L, Gin P, Weinstein M, Bensadoun A, et al. GPIHBP1: an endothelial cell molecule important for the lipolytic processing of chylomicrons. Curr Opin Lipidol. 2007;18:389-96 pubmed
  4. Adeyo O, Allan B, Barnes R, Goulbourne C, Tatar A, Tu Y, et al. Palmoplantar keratoderma along with neuromuscular and metabolic phenotypes in Slurp1-deficient mice. J Invest Dermatol. 2014;134:1589-1598 pubmed publisher
    ..Thus, Slurp1 deficiency in mice elicits metabolic and neuromuscular abnormalities in addition to PPK. ..
  5. Young S, Zechner R. Biochemistry and pathophysiology of intravascular and intracellular lipolysis. Genes Dev. 2013;27:459-84 pubmed publisher
    ..This review summarizes current views of lipolysis and highlights the relevance of this process to human disease. ..
  6. Goulbourne C, Gin P, Tatar A, Nobumori C, Hoenger A, Jiang H, et al. The GPIHBP1-LPL complex is responsible for the margination of triglyceride-rich lipoproteins in capillaries. Cell Metab. 2014;19:849-60 pubmed publisher
    ..Our studies show that GPIHBP1-bound LPL is the main determinant of TRL margination. ..
  7. Young S, Jung H, Coffinier C, Fong L. Understanding the roles of nuclear A- and B-type lamins in brain development. J Biol Chem. 2012;287:16103-10 pubmed publisher
    ..The relevance of lamins A and C in the brain remains unclear, but it is intriguing that prelamin A expression in the brain is low and is regulated by miR-9, a brain-specific microRNA. ..
  8. Allan C, Procaccia S, Tran D, Tu Y, Barnes R, Larsson M, et al. Palmoplantar Keratoderma in Slurp2-Deficient Mice. J Invest Dermatol. 2016;136:436-443 pubmed publisher
    ..Slurp2X(-/-) mice exhibited the same disease phenotypes. Thus, Slurp2 deficiency and Slurp1 deficiencies cause the same disease phenotypes. ..
  9. Fong L, Young S, Beigneux A, Bensadoun A, Oberer M, Jiang H, et al. GPIHBP1 and Plasma Triglyceride Metabolism. Trends Endocrinol Metab. 2016;27:455-469 pubmed publisher
    ..The discovery of GPIHBP1 has substantially revised our understanding of intravascular triglyceride metabolism but has also raised many new questions for future research. ..
  10. Hu X, Dallinga Thie G, Hovingh G, Chang S, Sandoval N, Dang T, et al. GPIHBP1 autoantibodies in a patient with unexplained chylomicronemia. J Clin Lipidol. 2017;11:964-971 pubmed publisher
    ..Additional studies in large lipid clinics will be helpful for better defining the frequency of this syndrome and for exploring the best strategies for treatment. ..
  11. request reprint
    Young S, Meta M, Yang S, Fong L. Prelamin A farnesylation and progeroid syndromes. J Biol Chem. 2006;281:39741-5 pubmed
    ..Also, administering an FTI to mouse models of HGPS and RD ameliorates the phenotypes of progeria. These studies have prompted interest in testing the efficacy of FTIs in children with HGPS. ..
  12. request reprint
    Young S, Fong L, Michaelis S. Prelamin A, Zmpste24, misshapen cell nuclei, and progeria--new evidence suggesting that protein farnesylation could be important for disease pathogenesis. J Lipid Res. 2005;46:2531-58 pubmed