Stephen Granville Young, M.D.

Department Address:
650 Charles E. Young Drive South
A2-237 CHS Bldg.
Los Angeles, CA 90095

Office Address:
695 Charles E. Young Drive South
GONDA 4506A
Los Angeles, CA 90095

Affiliations
Affiliations
Distinguished Professor, Medicine
Professor, Human Genetics
Member, Biochemistry, Biophysics & Structural Biology GPB Home Area, Molecular, Cellular & Integrative Physiology GPB Home Area
Faculty, Cardiology
Research Interests
We identified a GPI-anchored endothelial cell protein, GPIHBP1, that is required for the processing of triglyceride-rich lipoproteins (TRLs). We went on to show that GPIHBP1 binds lipoprotein lipase (LPL) in the interstitial spaces and shuttles it across endothelial cells to its site of action in the capillary lumen. In the absence of GPIHBP1, LPL remains mislocalized in the interstitial spaces and never reaches the capillary lumen. The mislocalization of LPL severely impairs the processing of TRLs in the plasma, causing severe hypertriglyceridemia. We have identified many GPIHBP1 mutations causing hypertriglyceridemia in humans; all of those interfere with the binding of LPL. Currently, we are working on mechanisms by which the fatty acid products of TRL processing move across capillary endothelial cells to vital tissues such as the heart. We also study the role of the nuclear lamin proteins in health and disease. Nuclear lamins are building blocks for the nuclear lamina, a structural scaffolding for the cell nucleus. Over the past few years, we elucidated the in vivo importance of the B-type lamins, lamin B1 and lamin B2, two key proteins of the nuclear lamina. We showed that both lamin B1 and lamin B2 are essential for the migration of neurons in the developing brain. We have also worked to develop strategies for treating genetic diseases caused by defective biogenesis of lamin A, another protein of the nuclear lamina.
Biography

Stephen Young grew up in Kansas and obtained an undergraduate degree in history from Princeton University. Following medical school at Washington University-St. Louis, he did internal medicine training at UCSF and cardiology training at UCSD. He then embarked on a career in basic science. He did postdoctoral research training in lipid metabolism at UCSD with Dr. Joseph Witztum and then worked at a UCSF-affiliated research institute for 17 years. Along the way, he became an expert in using genetically modified mice to investigate the gene function in health and disease. Currently, his laboratory studies an endothelial cell protein required for plasma triglyceride metabolism and diseases of the nuclear envelope. His laboratory has a strong track record in developing the careers of young scientists. Dr. Young has received multiple awards and is a member of the National Academy of Sciences.

Publications
Fong Loren G, Young Stephen G, Beigneux Anne P, Bensadoun André, Oberer Monika, Jiang Haibo, Ploug Michael GPIHBP1 and Plasma Triglyceride Metabolism. Trends in endocrinology and metabolism: TEM. 2016; .
Lee John M, Nobumori Chika, Tu Yiping, Choi Catherine, Yang Shao H, Jung Hea-Jin, Vickers Timothy A, Rigo Frank, Bennett C Frank, Young Stephen G, Fong Loren G Modulation of LMNA splicing as a strategy to treat prelamin A diseases. The Journal of clinical investigation. 2016; 126(4): 1592-602.
Dijk Wieneke, Beigneux Anne P, Larsson Mikael, Bensadoun André, Young Stephen G, Kersten Sander Angiopoietin-like 4 (ANGPTL4) promotes intracellular degradation of lipoprotein lipase in adipocytes. Journal of lipid research. 2016; 10(4): .
Vergnes Laurent, Chin Robert G, de Aguiar Vallim Thomas, Fong Loren G, Osborne Timothy F, Young Stephen G, Reue Karen SREBP-2-deficient and hypomorphic mice reveal roles for SREBP-2 in embryonic development and SREBP-1c expression. Journal of lipid research. 2016; 57(3): 410-21.
Mysling Simon, Kristensen Kristian Kølby, Larsson Mikael, Beigneux Anne P, Gårdsvoll Henrik, Fong Loren G, Bensadouen André, Jørgensen Thomas Jd, Young Stephen G, Ploug Michael The acidic domain of the endothelial membrane protein GPIHBP1 stabilizes lipoprotein lipase activity by preventing unfolding of its catalytic domain. eLife. 2016; 5(2): e12095.
Yang Shao H, Procaccia Shiri, Jung Hea-Jin, Nobumori Chika, Tatar Angelica, Tu Yiping, Bayguinov Yulia R, Hwang Sung Jin, Tran Deanna, Ward Sean M, Fong Loren G, Young Stephen G Mice that express farnesylated versions of prelamin A in neurons develop achalasia. Human molecular genetics. 2015; 24(10): 2826-40.
Rong Xin, Wang Bo, Dunham Merlow M, Hedde Per Niklas, Wong Jinny S, Gratton Enrico, Young Stephen G, Ford David A, Tontonoz Peter Lpcat3-dependent production of arachidonoyl phospholipids is a key determinant of triglyceride secretion. eLife. 2015; 4(1665): .
Beigneux Anne P, Fong Loren G, Bensadoun André, Davies Brandon S J, Oberer Monika, Gårdsvoll Henrik, Ploug Michael, Young Stephen G GPIHBP1 missense mutations often cause multimerization of GPIHBP1 and thereby prevent lipoprotein lipase binding. Circulation research. 2015; 116(4): 624-32.
Jung Hea-Jin, Tatar Angelica, Tu Yiping, Nobumori Chika, Yang Shao H, Goulbourne Chris N, Herrmann Harald, Fong Loren G, Young Stephen G An absence of nuclear lamins in keratinocytes leads to ichthyosis, defective epidermal barrier function, and intrusion of nuclear membranes and endoplasmic reticulum into the nuclear chromatin. Molecular and cellular biology. 2014; 34(24): 4534-44.
Hong Cynthia, Marshall Stephanie M, McDaniel Allison L, Graham Mark, Layne Joseph D, Cai Lei, Scotti Elena, Boyadjian Rima, Kim Jason, Chamberlain Brian T, Tangirala Rajendra K, Jung Michael E, Fong Loren, Lee Richard, Young Stephen G, Temel Ryan E, Tontonoz Peter The LXR-Idol axis differentially regulates plasma LDL levels in primates and mice. Cell metabolism. 2014; 20(5): 910-8.
Jiang Haibo, Goulbourne Chris N, Tatar Angelica, Turlo Kirsten, Wu Daniel, Beigneux Anne P, Grovenor Chris R M, Fong Loren G, Young Stephen G High-resolution imaging of dietary lipids in cells and tissues by NanoSIMS analysis. Journal of lipid research. 2014; 55(10): 2156-66.
Plengpanich Wanee, Young Stephen G, Khovidhunkit Weerapan, Bensadoun André, Karnman Hirankorn, Ploug Michael, Gårdsvoll Henrik, Leung Calvin S, Adeyo Oludotun, Larsson Mikael, Muanpetch Suwanna, Charoen Supannika, Fong Loren G, Niramitmahapanya Sathit, Beigneux Anne P Multimerization of glycosylphosphatidylinositol-anchored high density lipoprotein-binding protein 1 (GPIHBP1) and familial chylomicronemia from a serine-to-cysteine substitution in GPIHBP1 Ly6 domain. The Journal of biological chemistry. 2014; 289(28): 19491-9.
Adeyo Oludotun, Allan Bernard B, Barnes Richard H, Goulbourne Chris N, Tatar Angelica, Tu Yiping, Young Lorraine C, Weinstein Michael M, Tontonoz Peter, Fong Loren G, Beigneux Anne P, Young Stephen G Palmoplantar keratoderma along with neuromuscular and metabolic phenotypes in Slurp1-deficient mice. The Journal of investigative dermatology. 2014; 134(6): 1589-98.
Lee John M, Tu Yiping, Tatar Angelica, Wu Daniel, Nobumori Chika, Jung Hea-Jin, Yoshinaga Yuko, Coffinier Catherine, de Jong Pieter J, Fong Loren G, Young Stephen G Reciprocal knock-in mice to investigate the functional redundancy of lamin B1 and lamin B2. Molecular biology of the cell. 2014; 25(10): 1666-75.
Goulbourne Chris N, Gin Peter, Tatar Angelica, Nobumori Chika, Hoenger Andreas, Jiang Haibo, Grovenor Chris R M, Adeyo Oludotun, Esko Jeffrey D, Goldberg Ira J, Reue Karen, Tontonoz Peter, Bensadoun André, Beigneux Anne P, Young Stephen G, Fong Loren G The GPIHBP1-LPL complex is responsible for the margination of triglyceride-rich lipoproteins in capillaries. Cell metabolism. 2014; 19(5): 849-60.
Csaki Lauren S, Dwyer Jennifer R, Li Xia, Nguyen Michael H K, Dewald Jay, Brindley David N, Lusis Aldons J, Yoshinaga Yuko, de Jong Pieter, Fong Loren, Young Stephen G, Reue Karen Lipin-1 and lipin-3 together determine adiposity in vivo. Molecular metabolism. 2014; 3(2): 145-54.
Jung Hea-Jin, Tu Yiping, Yang Shao H, Tatar Angelica, Nobumori Chika, Wu Daniel, Young Stephen G, Fong Loren G New Lmna knock-in mice provide a molecular mechanism for the 'segmental aging' in Hutchinson-Gilford progeria syndrome. Human molecular genetics. 2014; 23(6): 1506-15.
Csaki Lauren S, Dwyer Jennifer R, Fong Loren G, Tontonoz Peter, Young Stephen G, Reue Karen Lipins, lipinopathies, and the modulation of cellular lipid storage and signaling. Progress in lipid research. 2013; 52(3): 305-16.
Jung Hea-Jin, Nobumori Chika, Goulbourne Chris N, Tu Yiping, Lee John M, Tatar Angelica, Wu Daniel, Yoshinaga Yuko, de Jong Pieter J, Coffinier Catherine, Fong Loren G, Young Stephen G Farnesylation of lamin B1 is important for retention of nuclear chromatin during neuronal migration. Proceedings of the National Academy of Sciences of the United States of America. 2013; 110(21): E1923-32.
Scotti Elena, Calamai Martino, Goulbourne Chris N, Zhang Li, Hong Cynthia, Lin Ron R, Choi Jinkuk, Pilch Paul F, Fong Loren G, Zou Peng, Ting Alice Y, Pavone Francesco S, Young Stephen G, Tontonoz Peter IDOL stimulates clathrin-independent endocytosis and multivesicular body-mediated lysosomal degradation of the low-density lipoprotein receptor. Molecular and cellular biology. 2013; 33(8): 1503-14.
Chen Xiao-Wei, Wang He, Bajaj Kanika, Zhang Pengcheng, Meng Zhuo-Xian, Ma Danjun, Bai Yongsheng, Liu Hui-Hui, Adams Elizabeth, Baines Andrea, Yu Genggeng, Sartor Maureen A, Zhang Bin, Yi Zhengping, Lin Jiandie, Young Stephen G, Schekman Randy, Ginsburg David SEC24A deficiency lowers plasma cholesterol through reduced PCSK9 secretion. eLife. 2013; 2(4): e00444.
Villanueva Claudio J, Vergnes Laurent, Wang Jiexin, Drew Brian G, Hong Cynthia, Tu Yiping, Hu Yan, Peng Xu, Xu Feng, Saez Enrique, Wroblewski Kevin, Hevener Andrea L, Reue Karen, Fong Loren G, Young Stephen G, Tontonoz Peter Adipose subtype-selective recruitment of TLE3 or Prdm16 by PPARγ specifies lipid storage versus thermogenic gene programs. Cell metabolism. 2013; 17(3): 423-35.
Young Stephen G, Zechner Rudolf Biochemistry and pathophysiology of intravascular and intracellular lipolysis. Genes & development. 2013; 27(5): 459-84.
Jung Hea-Jin, Lee John M, Yang Shao H, Young Stephen G, Fong Loren G Nuclear lamins in the brain - new insights into function and regulation. Molecular neurobiology. 2013; 47(1): 290-301.
Young Stephen G, Yang Shao H, Davies Brandon S J, Jung Hea-Jin, Fong Loren G Targeting protein prenylation in progeria. Science translational medicine. 2013; 5(171): 171ps3.
Davies Brandon S J, Goulbourne Chris N, Barnes Richard H, Turlo Kirsten A, Gin Peter, Vaughan Sue, Vaux David J, Bensadoun André, Beigneux Anne P, Fong Loren G, Young Stephen G Assessing mechanisms of GPIHBP1 and lipoprotein lipase movement across endothelial cells. Journal of lipid research. 2012; 53(12): 2690-7.
Zhang Li, Reue Karen, Fong Loren G, Young Stephen G, Tontonoz Peter Feedback regulation of cholesterol uptake by the LXR-IDOL-LDLR axis. Arteriosclerosis, thrombosis, and vascular biology. 2012; 32(11): 2541-6.
Dwyer Jennifer R, Donkor Jimmy, Zhang Peixiang, Csaki Lauren S, Vergnes Laurent, Lee Jessica M, Dewald Jay, Brindley David N, Atti Elisa, Tetradis Sotirios, Yoshinaga Yuko, De Jong Pieter J, Fong Loren G, Young Stephen G, Reue Karen Mouse lipin-1 and lipin-2 cooperate to maintain glycerolipid homeostasis in liver and aging cerebellum. Proceedings of the National Academy of Sciences of the United States of America. 2012; 109(37): E2486-95.
Gin Peter, Goulbourne Chris N, Adeyo Oludotun, Beigneux Anne P, Davies Brandon S J, Tat Shelly, Voss Constance V, Bensadoun André, Fong Loren G, Young Stephen G Chylomicronemia mutations yield new insights into interactions between lipoprotein lipase and GPIHBP1. Human molecular genetics. 2012; 21(13): 2961-72.
Chang Sandy Y, Hudon-Miller Sarah E, Yang Shao H, Jung Hea-Jin, Lee John M, Farber Emily, Subramanian Thangaiah, Andres Douglas A, Spielmann H Peter, Hrycyna Christine A, Young Stephen G, Fong Loren G Inhibitors of protein geranylgeranyltransferase-I lead to prelamin A accumulation in cells by inhibiting ZMPSTE24. Journal of lipid research. 2012; 53(6): 1176-82.
Young Stephen G, Jung Hea-Jin, Coffinier Catherine, Fong Loren G Understanding the roles of nuclear A- and B-type lamins in brain development. The Journal of biological chemistry. 2012; 287(20): 16103-10.
Young Stephen G, Fong Loren G Lowering plasma cholesterol by raising LDL receptors--revisited. The New England journal of medicine. 2012; 366(12): 1154-5.
Davies Brandon S J, Beigneux Anne P, Fong Loren G, Young Stephen G New wrinkles in lipoprotein lipase biology. Current opinion in lipidology. 2012; 23(1): 35-42.
Weinstein Michael M, Goulbourne Christopher N, Davies Brandon S J, Tu Yiping, Barnes Richard H, Watkins Steven M, Davis Ryan, Reue Karen, Tontonoz Peter, Beigneux Anne P, Fong Loren G, Young Stephen G Reciprocal metabolic perturbations in the adipose tissue and liver of GPIHBP1-deficient mice. Arteriosclerosis, thrombosis, and vascular biology. 2012; 32(2): 230-5.
Jung Hea-Jin, Coffinier Catherine, Choe Youngshik, Beigneux Anne P, Davies Brandon S J, Yang Shao H, Barnes Richard H, Hong Janet, Sun Tao, Pleasure Samuel J, Young Stephen G, Fong Loren G Regulation of prelamin A but not lamin C by miR-9, a brain-specific microRNA. Proceedings of the National Academy of Sciences of the United States of America. 2012; 109(7): E423-31.
Yang Shao H, Chang Sandy Y, Tu Yiping, Lawson Gregory W, Bergo Martin O, Fong Loren G, Young Stephen G Severe hepatocellular disease in mice lacking one or both CaaX prenyltransferases. Journal of lipid research. 2012; 53(1): 77-86.
Coffinier Catherine, Jung Hea-Jin, Nobumori Chika, Chang Sandy, Tu Yiping, Barnes Richard H, Yoshinaga Yuko, de Jong Pieter J, Vergnes Laurent, Reue Karen, Fong Loren G, Young Stephen G Deficiencies in lamin B1 and lamin B2 cause neurodevelopmental defects and distinct nuclear shape abnormalities in neurons. Molecular biology of the cell. 2011; 22(23): 4683-93.
Yang Shao H, Jung Hea-Jin, Coffinier Catherine, Fong Loren G, Young Stephen G Are B-type lamins essential in all mammalian cells?. Nucleus (Austin, Tex.). 2011; 2(6): 562-9.
Young Stephen G, Davies Brandon S J, Voss Constance V, Gin Peter, Weinstein Michael M, Tontonoz Peter, Reue Karen, Bensadoun André, Fong Loren G, Beigneux Anne P GPIHBP1, an endothelial cell transporter for lipoprotein lipase. Journal of lipid research. 2011; 52(11): 1869-84.
Yang Shao H, Chang Sandy Y, Yin Liya, Tu Yiping, Hu Yan, Yoshinaga Yuko, de Jong Pieter J, Fong Loren G, Young Stephen G An absence of both lamin B1 and lamin B2 in keratinocytes has no effect on cell proliferation or the development of skin and hair. Human molecular genetics. 2011; 20(18): 3537-44.
Beigneux Anne P, Davies Brandon S J, Tat Shelly, Chen Jenny, Gin Peter, Voss Constance V, Weinstein Michael M, Bensadoun André, Pullinger Clive R, Fong Loren G, Young Stephen G Assessing the role of the glycosylphosphatidylinositol-anchored high density lipoprotein-binding protein 1 (GPIHBP1) three-finger domain in binding lipoprotein lipase. The Journal of biological chemistry. 2011; 286(22): 19735-43.
Im Seung-Soon, Yousef Leyla, Blaschitz Christoph, Liu Janet Z, Edwards Robert A, Young Stephen G, Raffatellu Manuela, Osborne Timothy F Linking lipid metabolism to the innate immune response in macrophages through sterol regulatory element binding protein-1a. Cell metabolism. 2011; 13(5): 540-9.
Voss Constance V, Davies Brandon S J, Tat Shelly, Gin Peter, Fong Loren G, Pelletier Christopher, Mottler Charlene D, Bensadoun André, Beigneux Anne P, Young Stephen G Mutations in lipoprotein lipase that block binding to the endothelial cell transporter GPIHBP1. Proceedings of the National Academy of Sciences of the United States of America. 2011; 108(19): 7980-4.
Scotti Elena, Hong Cynthia, Yoshinaga Yuko, Tu Yiping, Hu Yan, Zelcer Noam, Boyadjian Rima, de Jong Pieter J, Young Stephen G, Fong Loren G, Tontonoz Peter Targeted disruption of the idol gene alters cellular regulation of the low-density lipoprotein receptor by sterols and liver x receptor agonists. Molecular and cellular biology. 2011; 31(9): 1885-93.
Feig Jonathan E, Parathath Sajesh, Rong James X, Mick Stephanie L, Vengrenyuk Yuliya, Grauer Lisa, Young Stephen G, Fisher Edward A Reversal of hyperlipidemia with a genetic switch favorably affects the content and inflammatory state of macrophages in atherosclerotic plaques. Circulation. 2011; 123(9): 989-98.
Yang Shao H, Chang Sandy Y, Ren Shuxun, Wang Yibin, Andres Douglas A, Spielmann H Peter, Fong Loren G, Young Stephen G Absence of progeria-like disease phenotypes in knock-in mice expressing a non-farnesylated version of progerin. Human molecular genetics. 2011; 20(3): 436-44.
Gin Peter, Beigneux Anne P, Voss Constance, Davies Brandon S J, Beckstead Jennifer A, Ryan Robert O, Bensadoun André, Fong Loren G, Young Stephen G Binding preferences for GPIHBP1, a glycosylphosphatidylinositol-anchored protein of capillary endothelial cells. Arteriosclerosis, thrombosis, and vascular biology. 2011; 31(1): 176-82.
Vergnes Laurent, Chin Robert, Young Stephen G, Reue Karen Heart-type fatty acid-binding protein is essential for efficient brown adipose tissue fatty acid oxidation and cold tolerance. The Journal of biological chemistry. 2011; 286(1): 380-90.
Davies Brandon Sj, Coffinier Catherine, Yang Shao H, Barnes Richard H, Jung Hea-Jin, Young Stephen G, Fong Loren G Investigating the purpose of prelamin A processing. Nucleus (Austin, Tex.). 2011; 2(1): 4-9.
Bishop Joseph R, Passos-Bueno Maria Rita, Fong Loren, Stanford Kristin I, Gonzales Jon C, Yeh Erika, Young Stephen G, Bensadoun Andre, Witztum Joseph L, Esko Jeffrey D, Moulton Karen S Deletion of the basement membrane heparan sulfate proteoglycan type XVIII collagen causes hypertriglyceridemia in mice and humans. PloS one. 2010; 5(11): e13919.
Olafsen Tove, Young Stephen G, Davies Brandon S J, Beigneux Anne P, Kenanova Vania E, Voss Constance, Young Glen, Wong Koon-Pong, Barnes Richard H, Tu Yiping, Weinstein Michael M, Nobumori Chika, Huang Sung-Cheng, Goldberg Ira J, Bensadoun André, Wu Anna M, Fong Loren G Unexpected expression pattern for glycosylphosphatidylinositol-anchored HDL-binding protein 1 (GPIHBP1) in mouse tissues revealed by positron emission tomography scanning. The Journal of biological chemistry. 2010; 285(50): 39239-48.
Weinstein Michael M, Tu Yiping, Beigneux Anne P, Davies Brandon S J, Gin Peter, Voss Constance, Walzem Rosemary L, Reue Karen, Tontonoz Peter, Bensadoun André, Fong Loren G, Young Stephen G Cholesterol intake modulates plasma triglyceride levels in glycosylphosphatidylinositol HDL-binding protein 1-deficient mice. Arteriosclerosis, thrombosis, and vascular biology. 2010; 30(11): 2106-13.
Hernandez Lidia, Roux Kyle J, Wong Esther Sook Miin, Mounkes Leslie C, Mutalif Rafidah, Navasankari Raju, Rai Bina, Cool Simon, Jeong Jae-Wook, Wang Honghe, Lee Hyun-Shik, Kozlov Serguei, Grunert Martin, Keeble Thomas, Jones C Michael, Meta Margarita D, Young Stephen G, Daar Ira O, Burke Brian, Perantoni Alan O, Stewart Colin L Functional coupling between the extracellular matrix and nuclear lamina by Wnt signaling in progeria. Developmental cell. 2010; 19(3): 413-25.
Coffinier Catherine, Fong Loren G, Young Stephen G LINCing lamin B2 to neuronal migration: growing evidence for cell-specific roles of B-type lamins. Nucleus (Austin, Tex.). 2010; 1(5): 407-11.
Davies Brandon S J, Barnes Richard H, Tu Yiping, Ren Shuxun, Andres Douglas A, Spielmann H Peter, Lammerding Jan, Wang Yibin, Young Stephen G, Fong Loren G An accumulation of non-farnesylated prelamin A causes cardiomyopathy but not progeria. Human molecular genetics. 2010; 19(13): 2682-94.
Coffinier Catherine, Jung Hea-Jin, Li Ziwei, Nobumori Chika, Yun Ui Jeong, Farber Emily A, Davies Brandon S, Weinstein Michael M, Yang Shao H, Lammerding Jan, Farahani Javad N, Bentolila Laurent A, Fong Loren G, Young Stephen G Direct synthesis of lamin A, bypassing prelamin a processing, causes misshapen nuclei in fibroblasts but no detectable pathology in mice. The Journal of biological chemistry. 2010; 285(27): 20818-26.
Davies Brandon S J, Beigneux Anne P, Barnes Richard H, Tu Yiping, Gin Peter, Weinstein Michael M, Nobumori Chika, Nyrén Rakel, Goldberg Ira, Olivecrona Gunilla, Bensadoun André, Young Stephen G, Fong Loren G GPIHBP1 is responsible for the entry of lipoprotein lipase into capillaries. Cell metabolism. 2010; 12(1): 42-52.
Weinstein MM, Yin L, Beigneux AP, Davies BS, Gin P, Estrada K, Melford K, Bishop JR, Esko JD, Dallinga-Thie GM, Fong LG, Bensadoun A, Young SG. Abnormal patterns of lipoprotein lipase release into the plasma in GPIHPB1-deficient mice . J. Biol. Chem. 2010; 12: 34511–34518.
Coffinier C, Hudon SE, Farber EA, Chang SY, Hrycyna CA, Young SG, Fong LG. HIV protease inhibitors block the zinc metalloproteinase ZMPSTE24 and lead to an accumulation of prelamin A in cells. Proc. Natl. Acad. Sci. USA 2010; 104: 13432-13437.
Olivecrona Gunilla, Ehrenborg Ewa, Semb Henrik, Makoveichuk Elena, Lindberg Anna, Hayden Michael R, Gin Peter, Davies Brandon S J, Weinstein Michael M, Fong Loren G, Beigneux Anne P, Young Stephen G, Olivecrona Thomas, Hernell Olle Mutation of conserved cysteines in the Ly6 domain of GPIHBP1 in familial chylomicronemia. Journal of lipid research. 2010; 51(6): 1535-45.
Franssen Remco, Young Stephen G, Peelman Frank, Hertecant Jozef, Sierts Jeroen A, Schimmel Alinda W M, Bensadoun André, Kastelein John J P, Fong Loren G, Dallinga-Thie Geesje M, Beigneux Anne P Chylomicronemia with low postheparin lipoprotein lipase levels in the setting of GPIHBP1 defects. Circulation. Cardiovascular genetics. 2010; 3(2): 169-78.
Lee Roger, Chang Sandy Y, Trinh Hung, Tu Yiping, White Andrew C, Davies Brandon S J, Bergo Martin O, Fong Loren G, Lowry William E, Young Stephen G Genetic studies on the functional relevance of the protein prenyltransferases in skin keratinocytes. Human molecular genetics. 2010; 19(8): 1603-17.
Coffinier Catherine, Chang Sandy Y, Nobumori Chika, Tu Yiping, Farber Emily A, Toth Julia I, Fong Loren G, Young Stephen G Abnormal development of the cerebral cortex and cerebellum in the setting of lamin B2 deficiency. Proceedings of the National Academy of Sciences of the United States of America. 2010; 107(11): 5076-81.
Yang Shao H, Chang Sandy Y, Andres Douglas A, Spielmann H Peter, Young Stephen G, Fong Loren G Assessing the efficacy of protein farnesyltransferase inhibitors in mouse models of progeria. Journal of lipid research. 2010; 51(2): 400-5.
Weinstein Michael M, Yin Liya, Tu Yiping, Wang Xuping, Wu Xiaohui, Castellani Lawrence W, Walzem Rosemary L, Lusis Aldons J, Fong Loren G, Beigneux Anne P, Young Stephen G Chylomicronemia elicits atherosclerosis in mice--brief report. Arteriosclerosis, thrombosis, and vascular biology. 2010; 30(1): 20-3.
Beigneux Anne P, Gin Peter, Davies Brandon S J, Weinstein Michael M, Bensadoun André, Fong Loren G, Young Stephen G Highly conserved cysteines within the Ly6 domain of GPIHBP1 are crucial for the binding of lipoprotein lipase. The Journal of biological chemistry. 2009; 284(44): 30240-7.
Im Seung-Soon, Hammond Linda E, Yousef Leyla, Nugas-Selby Cherryl, Shin Dong-Ju, Seo Young-Kyo, Fong Loren G, Young Stephen G, Osborne Timothy F Sterol regulatory element binding protein 1a regulates hepatic fatty acid partitioning by activating acetyl coenzyme A carboxylase 2. Molecular and cellular biology. 2009; 29(17): 4864-72.
Fong Loren G, Vickers Timothy A, Farber Emily A, Choi Christine, Yun Ui Jeong, Hu Yan, Yang Shao H, Coffinier Catherine, Lee Roger, Yin Liya, Davies Brandon S J, Andres Douglas A, Spielmann H Peter, Bennett C Frank, Young Stephen G Activating the synthesis of progerin, the mutant prelamin A in Hutchinson-Gilford progeria syndrome, with antisense oligonucleotides. Human molecular genetics. 2009; 18(13): 2462-71.
Worman Howard J, Fong Loren G, Muchir Antoine, Young Stephen G Laminopathies and the long strange trip from basic cell biology to therapy. The Journal of clinical investigation. 2009; 119(7): 1825-36.
Park Kye Won, Waki Hironori, Kim Woo-Kyun, Davies Brandon S J, Young Stephen G, Parhami Farhad, Tontonoz Peter The small molecule phenamil induces osteoblast differentiation and mineralization. Molecular and cellular biology. 2009; 29(14): 3905-14.
Lee R, Chang SY, Trinh H, White AC, Bergo MO, Fong LG, Lowry WE, and Young SG. Genetic Analysis of the Importance of the Protein Prenyltransferases in Skin Keratinocytes. Human Molecular Gen 2009; (19): 1603-1617.
Beigneux AP, Franssen R, Bensadoun A, Gin P, Melford K, Peter J, Walzem RL, Weinstein MM, Davies BS, Kuivenhoven JA, Kastelein JJ, Fong LG, Dallinga-Thie GM, Young SG. Chylomicronemia with a Mutant GPIHBP1 (Q115P) that Cannot Bind Lipoprotein Lipase. . Arterioscler Thromb Vasc Biol 2009; (29): 956-962.
Yang Shao H, Bergo Martin O, Farber Emily, Qiao Xin, Fong Loren G, Young Stephen G Caution! Analyze transcripts from conditional knockout alleles. Transgenic research. 2009; 18(3): 483-9.
Beigneux Anne P, Weinstein Michael M, Davies Brandon S J, Gin Peter, Bensadoun André, Fong Loren G, Young Stephen G GPIHBP1 and lipolysis: an update. Current opinion in lipidology. 2009; 20(3): 211-6.
Miller Jordan D, Weiss Robert M, Serrano Kristine M, Brooks Robert M, Berry Christopher J, Zimmerman Kathy, Young Stephen G, Heistad Donald D Lowering plasma cholesterol levels halts progression of aortic valve disease in mice. Circulation. 2009; 119(20): 2693-701.
Beigneux Anne P, Davies Brandon S J, Bensadoun André, Fong Loren G, Young Stephen G GPIHBP1, a GPI-anchored protein required for the lipolytic processing of triglyceride-rich lipoproteins. Journal of lipid research. 2009; 50 Suppl(20): S57-62.
Davies Brandon S J, Fong Loren G, Yang Shao H, Coffinier Catherine, Young Stephen G The posttranslational processing of prelamin A and disease. Annual review of genomics and human genetics. 2009; 10(20): 153-74.
Davies Brandon S J, Yang Shao H, Farber Emily, Lee Roger, Buck Suzanne B, Andres Douglas A, Spielmann H Peter, Agnew Brian J, Tamanoi Fuyuhiko, Fong Loren G, Young Stephen G Increasing the length of progerin's isoprenyl anchor does not worsen bone disease or survival in mice with Hutchinson-Gilford progeria syndrome. Journal of lipid research. 2009; 50(1): 126-34.
Weinstein Michael M, Yin Liya, Beigneux Anne P, Davies Brandon S J, Gin Peter, Estrada Kristine, Melford Kristan, Bishop Joseph R, Esko Jeffrey D, Dallinga-Thie Geesje M, Fong Loren G, Bensadoun André, Young Stephen G Abnormal patterns of lipoprotein lipase release into the plasma in GPIHBP1-deficient mice. The Journal of biological chemistry. 2008; 283(50): 34511-8.
Davies Brandon S J, Waki Hironori, Beigneux Anne P, Farber Emily, Weinstein Michael M, Wilpitz Damien C, Tai Li-Jung, Evans Ronald M, Fong Loren G, Tontonoz Peter, Young Stephen G The expression of GPIHBP1, an endothelial cell binding site for lipoprotein lipase and chylomicrons, is induced by peroxisome proliferator-activated receptor-gamma. Molecular endocrinology (Baltimore, Md.). 2008; 22(11): 2496-504.
Yang SH, Andres DA, Spielmann HP, Young SG, and Fong LG. Progerin elicits disease phenotypes of progeria in mice whether or not it is farnesylated. J. Clin. Invest 2008; 118: 3291-3300.
Gin P, Yin L, Davies BSJ, Weinstein MM, Ryan RO, Bensadoun A, Fong LG, Young SG, and Beigneux AP The acidic domain of GPIHBP1 is important for the binding of lipoprotein lipase and chylomicrons. J Biol. Chem 2008; 283: 29554-29562.
Beigneux Anne P, Gin Peter, Davies Brandon S J, Weinstein Michael M, Bensadoun André, Ryan Robert O, Fong Loren G, Young Stephen G Glycosylation of Asn-76 in mouse GPIHBP1 is critical for its appearance on the cell surface and the binding of chylomicrons and lipoprotein lipase. Journal of lipid research. 2008; 49(6): 1312-21.
Coffinier Catherine, Hudon Sarah E, Lee Roger, Farber Emily A, Nobumori Chika, Miner Jeffrey H, Andres Douglas A, Spielmann H Peter, Hrycyna Christine A, Fong Loren G, Young Stephen G A potent HIV protease inhibitor, darunavir, does not inhibit ZMPSTE24 or lead to an accumulation of farnesyl-prelamin A in cells. The Journal of biological chemistry. 2008; 283(15): 9797-804.
Gin Peter, Beigneux Anne P, Davies Brandon, Young Madeline F, Ryan Robert O, Bensadoun André, Fong Loren G, Young Stephen G Normal binding of lipoprotein lipase, chylomicrons, and apo-AV to GPIHBP1 containing a G56R amino acid substitution. Biochimica et biophysica acta. 2007; 1771(12): 1464-8.
Young Stephen G, Davies Brandon S J, Fong Loren G, Gin Peter, Weinstein Michael M, Bensadoun André, Beigneux Anne P GPIHBP1: an endothelial cell molecule important for the lipolytic processing of chylomicrons. Current opinion in lipidology. 2007; 18(4): 389-96.
Coffinier Catherine, Hudon Sarah E, Farber Emily A, Chang Sandy Y, Hrycyna Christine A, Young Stephen G, Fong Loren G HIV protease inhibitors block the zinc metalloproteinase ZMPSTE24 and lead to an accumulation of prelamin A in cells. Proceedings of the National Academy of Sciences of the United States of America. 2007; 104(33): 13432-7.
Beigneux AP, Davies BS, Gin P, Weinstein MM, Farber E, Qiao X, Peale F, Bunting S, Walzem RL, Wong JS, Blaner WS, Ding ZM, Melford K, Wongsiriroj N, Shu X, de Sauvage F, Ryan RO, Fong LG, Bensadoun A, Young SG. Glycosylphosphatidylinositol-anchored high-density lipoprotein-binding protein 1 plays a critical role in the lipolytic processing of chylomicrons. Cell Metabolism 2007; 5: 279–291.
Allan Christopher M, Jung Cris J, Larsson Mikael, Heizer Patrick J, Tu Yiping, Sandoval Norma P, Dang Tiffany Ly P, Jung Rachel S, Beigneux Anne P, de Jong Pieter J, Fong Loren G, Young Stephen G Mutating a conserved cysteine in GPIHBP1 reduces amounts of GPIHBP1 in capillaries and abolishes LPL binding. Journal of lipid research. 2017; 58(7): 1453-1461.
Beigneux Anne P, Miyashita Kazuya, Ploug Michael, Blom Dirk J, Ai Masumi, Linton MacRae F, Khovidhunkit Weerapan, Dufour Robert, Garg Abhimanyu, McMahon Maureen A, Pullinger Clive R, Sandoval Norma P, Hu Xuchen, Allan Christopher M, Larsson Mikael, Machida Tetsuo, Murakami Masami, Reue Karen, Tontonoz Peter, Goldberg Ira J, Moulin Philippe, Charrière Sybil, Fong Loren G, Nakajima Katsuyuki, Young Stephen G Autoantibodies against GPIHBP1 as a Cause of Hypertriglyceridemia. The New England journal of medicine. 2017; 376(17): 1647-1658.
Tu Yiping, Sánchez-Iglesias Sofía, Araújo-Vilar David, Fong Loren G, Young Stephen G LMNA missense mutations causing familial partial lipodystrophy do not lead to an accumulation of prelamin A. Nucleus (Austin, Tex.). 2016; 7(5): 512-521.
Allan Christopher M, Larsson Mikael, Hu Xuchen, He Cuiwen, Jung Rachel S, Mapar Alaleh, Voss Constance, Miyashita Kazuya, Machida Tetsuo, Murakami Masami, Nakajima Katsuyuki, Bensadoun André, Ploug Michael, Fong Loren G, Young Stephen G, Beigneux Anne P An LPL-specific monoclonal antibody, 88B8, that abolishes the binding of LPL to GPIHBP1. Journal of lipid research. 2016; 57(10): 1889-1898.
Wang Jiexin, Rajbhandari Prashant, Damianov Andrey, Han Areum, Sallam Tamer, Waki Hironori, Villanueva Claudio J, Lee Stephen D, Nielsen Ronni, Mandrup Susanne, Reue Karen, Young Stephen G, Whitelegge Julian, Saez Enrique, Black Douglas L, Tontonoz Peter RNA-binding protein PSPC1 promotes the differentiation-dependent nuclear export of adipocyte RNAs. The Journal of clinical investigation. 2017; 127(3): 987-1004.
He Cuiwen, Hu Xuchen, Jung Rachel S, Larsson Mikael, Tu Yiping, Duarte-Vogel Sandra, Kim Paul, Sandoval Norma P, Price Tara R, Allan Christopher M, Raney Brian, Jiang Haibo, Bensadoun André, Walzem Rosemary L, Kuo Richard I, Beigneux Anne P, Fong Loren G, Young Stephen G Lipoprotein lipase reaches the capillary lumen in chickens despite an apparent absence of GPIHBP1. JCI insight. 2017; 2(20): 1134-1136.
He Cuiwen, Hu Xuchen, Jung Rachel S, Weston Thomas A, Sandoval Norma P, Tontonoz Peter, Kilburn Matthew R, Fong Loren G, Young Stephen G, Jiang Haibo High-resolution imaging and quantification of plasma membrane cholesterol by NanoSIMS. Proceedings of the National Academy of Sciences of the United States of America. 2017; 114(8): 2000-2005.
Hu Xuchen, Dallinga-Thie Geesje M, Hovingh G Kees, Chang Sandy Y, Sandoval Norma P, Dang Tiffany Ly P, Fukamachi Isamu, Miyashita Kazuya, Nakajima Katsuyuki, Murakami Masami, Fong Loren G, Ploug Michael, Young Stephen G, Beigneux Anne P GPIHBP1 autoantibodies in a patient with unexplained chylomicronemia. Journal of clinical lipidology. 2017; 11(4): 964-971.
Hu Xuchen, Sleeman Mark W, Miyashita Kazuya, Linton MacRae F, Allan Christopher M, He Cuiwen, Larsson Mikael, Tu Yiping, Sandoval Norma P, Jung Rachel S, Mapar Alaleh, Machida Tetsuo, Murakami Masami, Nakajima Katsuyuki, Ploug Michael, Fong Loren G, Young Stephen G, Beigneux Anne P Monoclonal antibodies that bind to the Ly6 domain of GPIHBP1 abolish the binding of LPL. Journal of lipid research. 2017; 58(1): 208-215.
Allan Christopher M, Larsson Mikael, Jung Rachel S, Ploug Michael, Bensadoun André, Beigneux Anne P, Fong Loren G, Young Stephen G Mobility of "HSPG-bound" LPL explains how LPL is able to reach GPIHBP1 on capillaries. Journal of lipid research. 2017; 58(1): 216-225.
Mysling Simon, Kristensen Kristian Kølby, Larsson Mikael, Kovrov Oleg, Bensadouen André, Jørgensen Thomas Jd, Olivecrona Gunilla, Young Stephen G, Ploug Michael The angiopoietin-like protein ANGPTL4 catalyzes unfolding of the hydrolase domain in lipoprotein lipase and the endothelial membrane protein GPIHBP1 counteracts this unfolding. eLife. 2016; 5(1): 216-225.