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DYT1 Dystonia Patient-Derived Fibroblasts Have Increased Deformability and Susceptibility to Damage by Mechanical Forces.

Gill NK, Ly C, Kim PH, Saunders CA, Fong LG, Young SG, Luxton GWG, Rowat AC.

Front Cell Dev Biol. 2019 Jun 26;7:103. doi: 10.3389/fcell.2019.00103. eCollection 2019.


GPIHBP1 and Lipoprotein Lipase, Partners in Plasma Triglyceride Metabolism.

Young SG, Fong LG, Beigneux AP, Allan CM, He C, Jiang H, Nakajima K, Meiyappan M, Birrane G, Ploug M.

Cell Metab. 2019 Jul 2;30(1):51-65. doi: 10.1016/j.cmet.2019.05.023. Review.


GPIHBP1 expression in gliomas promotes utilization of lipoprotein-derived nutrients.

Hu X, Matsumoto K, Jung RS, Weston TA, Heizer PJ, He C, Sandoval NP, Allan CM, Tu Y, Vinters HV, Liau LM, Ellison RM, Morales JE, Baufeld LJ, Bayley NA, He L, Betsholtz C, Beigneux AP, Nathanson DA, Gerhardt H, Young SG, Fong LG, Jiang H.

Elife. 2019 Jun 6;8. pii: e47178. doi: 10.7554/eLife.47178.


Lipoprotein lipase is active as a monomer.

Beigneux AP, Allan CM, Sandoval NP, Cho GW, Heizer PJ, Jung RS, Stanhope KL, Havel PJ, Birrane G, Meiyappan M, Gill JE 4th, Murakami M, Miyashita K, Nakajima K, Ploug M, Fong LG, Young SG.

Proc Natl Acad Sci U S A. 2019 Mar 26;116(13):6319-6328. doi: 10.1073/pnas.1900983116. Epub 2019 Mar 8.


An upstream enhancer regulates Gpihbp1 expression in a tissue-specific manner.

Allan CM, Heizer PJ, Tu Y, Sandoval NP, Jung RS, Morales JE, Sajti E, Troutman TD, Saunders TL, Cusanovich DA, Beigneux AP, Romanoski CE, Fong LG, Young SG.

J Lipid Res. 2019 Apr;60(4):869-879. doi: 10.1194/jlr.M091322. Epub 2018 Dec 31.


Structure of the lipoprotein lipase-GPIHBP1 complex that mediates plasma triglyceride hydrolysis.

Birrane G, Beigneux AP, Dwyer B, Strack-Logue B, Kristensen KK, Francone OL, Fong LG, Mertens HDT, Pan CQ, Ploug M, Young SG, Meiyappan M.

Proc Natl Acad Sci U S A. 2019 Jan 29;116(5):1723-1732. doi: 10.1073/pnas.1817984116. Epub 2018 Dec 17.


Lipin 2/3 phosphatidic acid phosphatases maintain phospholipid homeostasis to regulate chylomicron synthesis.

Zhang P, Csaki LS, Ronquillo E, Baufeld LJ, Lin JY, Gutierrez A, Dwyer JR, Brindley DN, Fong LG, Tontonoz P, Young SG, Reue K.

J Clin Invest. 2019 Jan 2;129(1):281-295. doi: 10.1172/JCI122595. Epub 2018 Dec 3.


Prelamin A causes aberrant myonuclear arrangement and results in muscle fiber weakness.

Levy Y, Ross JA, Niglas M, Snetkov VA, Lynham S, Liao CY, Puckelwartz MJ, Hsu YM, McNally EM, Alsheimer M, Harridge SD, Young SG, Fong LG, Español Y, Lopez-Otin C, Kennedy BK, Lowe DA, Ochala J.

JCI Insight. 2018 Oct 4;3(19). pii: 120920. doi: 10.1172/jci.insight.120920.


Disrupting the LINC complex in smooth muscle cells reduces aortic disease in a mouse model of Hutchinson-Gilford progeria syndrome.

Kim PH, Luu J, Heizer P, Tu Y, Weston TA, Chen N, Lim C, Li RL, Lin PY, Dunn JCY, Hodzic D, Young SG, Fong LG.

Sci Transl Med. 2018 Sep 26;10(460). pii: eaat7163. doi: 10.1126/scitranslmed.aat7163.


Fibroblasts lacking nuclear lamins do not have nuclear blebs or protrusions but nevertheless have frequent nuclear membrane ruptures.

Chen NY, Kim P, Weston TA, Edillo L, Tu Y, Fong LG, Young SG.

Proc Natl Acad Sci U S A. 2018 Oct 2;115(40):10100-10105. doi: 10.1073/pnas.1812622115. Epub 2018 Sep 17.


NanoSIMS imaging reveals unexpected heterogeneity in nutrient uptake by brown adipocytes.

He C, Hu X, Weston TA, Jung RS, Heizer P, Tu Y, Ellison R, Matsumoto K, Gerhardt H, Tontonoz P, Fong LG, Young SG, Jiang H.

Biochem Biophys Res Commun. 2018 Oct 12;504(4):899-902. doi: 10.1016/j.bbrc.2018.09.051. Epub 2018 Sep 15.


Macrophages release plasma membrane-derived particles rich in accessible cholesterol.

He C, Hu X, Weston TA, Jung RS, Sandhu J, Huang S, Heizer P, Kim J, Ellison R, Xu J, Kilburn M, Bensinger SJ, Riezman H, Tontonoz P, Fong LG, Jiang H, Young SG.

Proc Natl Acad Sci U S A. 2018 Sep 4;115(36):E8499-E8508. doi: 10.1073/pnas.1810724115. Epub 2018 Aug 20.


A disordered acidic domain in GPIHBP1 harboring a sulfated tyrosine regulates lipoprotein lipase.

Kristensen KK, Midtgaard SR, Mysling S, Kovrov O, Hansen LB, Skar-Gislinge N, Beigneux AP, Kragelund BB, Olivecrona G, Young SG, Jørgensen TJD, Fong LG, Ploug M.

Proc Natl Acad Sci U S A. 2018 Jun 26;115(26):E6020-E6029. doi: 10.1073/pnas.1806774115. Epub 2018 Jun 13.


NanoSIMS Analysis of Intravascular Lipolysis and Lipid Movement across Capillaries and into Cardiomyocytes.

He C, Weston TA, Jung RS, Heizer P, Larsson M, Hu X, Allan CM, Tontonoz P, Reue K, Beigneux AP, Ploug M, Holme A, Kilburn M, Guagliardo P, Ford DA, Fong LG, Young SG, Jiang H.

Cell Metab. 2018 May 1;27(5):1055-1066.e3. doi: 10.1016/j.cmet.2018.03.017.


Impaired thermogenesis and sharp increases in plasma triglyceride levels in GPIHBP1-deficient mice during cold exposure.

Larsson M, Allan CM, Heizer PJ, Tu Y, Sandoval NP, Jung RS, Walzem RL, Beigneux AP, Young SG, Fong LG.

J Lipid Res. 2018 Apr;59(4):706-713. doi: 10.1194/jlr.M083832. Epub 2018 Feb 15.


IL-10 Signaling Remodels Adipose Chromatin Architecture to Limit Thermogenesis and Energy Expenditure.

Rajbhandari P, Thomas BJ, Feng AC, Hong C, Wang J, Vergnes L, Sallam T, Wang B, Sandhu J, Seldin MM, Lusis AJ, Fong LG, Katz M, Lee R, Young SG, Reue K, Smale ST, Tontonoz P.

Cell. 2018 Jan 11;172(1-2):218-233.e17. doi: 10.1016/j.cell.2017.11.019. Epub 2017 Dec 14.


Lipoprotein lipase reaches the capillary lumen in chickens despite an apparent absence of GPIHBP1.

He C, Hu X, Jung RS, Larsson M, Tu Y, Duarte-Vogel S, Kim P, Sandoval NP, Price TR, Allan CM, Raney B, Jiang H, Bensadoun A, Walzem RL, Kuo RI, Beigneux AP, Fong LG, Young SG.

JCI Insight. 2017 Oct 19;2(20). pii: 96783. doi: 10.1172/jci.insight.96783.


Palmoplantar keratoderma in Slurp1/Slurp2 double-knockout mice.

Allan CM, Heizer PJ, Jung CJ, Tu Y, Tran D, Young LC, Fong LG, de Jong PJ, Beigneux AP, Young SG.

J Dermatol Sci. 2018 Jan;89(1):85-87. doi: 10.1016/j.jdermsci.2017.08.014. Epub 2017 Sep 28. No abstract available.


Apolipoprotein C-III inhibits triglyceride hydrolysis by GPIHBP1-bound LPL.

Larsson M, Allan CM, Jung RS, Heizer PJ, Beigneux AP, Young SG, Fong LG.

J Lipid Res. 2017 Sep;58(9):1893-1902. doi: 10.1194/jlr.M078220. Epub 2017 Jul 10.


GPIHBP1 autoantibodies in a patient with unexplained chylomicronemia.

Hu X, Dallinga-Thie GM, Hovingh GK, Chang SY, Sandoval NP, Dang TLP, Fukamachi I, Miyashita K, Nakajima K, Murakami M, Fong LG, Ploug M, Young SG, Beigneux AP.

J Clin Lipidol. 2017 Jul - Aug;11(4):964-971. doi: 10.1016/j.jacl.2017.05.017. Epub 2017 Jun 13.


Mutating a conserved cysteine in GPIHBP1 reduces amounts of GPIHBP1 in capillaries and abolishes LPL binding.

Allan CM, Jung CJ, Larsson M, Heizer PJ, Tu Y, Sandoval NP, Dang TLP, Jung RS, Beigneux AP, de Jong PJ, Fong LG, Young SG.

J Lipid Res. 2017 Jul;58(7):1453-1461. doi: 10.1194/jlr.M076943. Epub 2017 May 5.


A hypomorphic Egfr allele does not ameliorate the palmoplantar keratoderma caused by SLURP1 deficiency.

Allan CM, Tran D, Tu Y, Heizer PJ, Young LC, Fong LG, Beigneux AP, Young SG.

Exp Dermatol. 2017 Nov;26(11):1134-1136. doi: 10.1111/exd.13363. Epub 2017 Jul 27.


Autoantibodies against GPIHBP1 as a Cause of Hypertriglyceridemia.

Beigneux AP, Miyashita K, Ploug M, Blom DJ, Ai M, Linton MF, Khovidhunkit W, Dufour R, Garg A, McMahon MA, Pullinger CR, Sandoval NP, Hu X, Allan CM, Larsson M, Machida T, Murakami M, Reue K, Tontonoz P, Goldberg IJ, Moulin P, Charrière S, Fong LG, Nakajima K, Young SG.

N Engl J Med. 2017 Apr 27;376(17):1647-1658. doi: 10.1056/NEJMoa1611930. Epub 2017 Apr 5.


High-resolution imaging and quantification of plasma membrane cholesterol by NanoSIMS.

He C, Hu X, Jung RS, Weston TA, Sandoval NP, Tontonoz P, Kilburn MR, Fong LG, Young SG, Jiang H.

Proc Natl Acad Sci U S A. 2017 Feb 21;114(8):2000-2005. doi: 10.1073/pnas.1621432114. Epub 2017 Feb 6.


Monoclonal antibodies that bind to the Ly6 domain of GPIHBP1 abolish the binding of LPL.

Hu X, Sleeman MW, Miyashita K, Linton MF, Allan CM, He C, Larsson M, Tu Y, Sandoval NP, Jung RS, Mapar A, Machida T, Murakami M, Nakajima K, Ploug M, Fong LG, Young SG, Beigneux AP.

J Lipid Res. 2017 Jan;58(1):208-215. doi: 10.1194/jlr.M072462. Epub 2016 Nov 15.


LMNA missense mutations causing familial partial lipodystrophy do not lead to an accumulation of prelamin A.

Tu Y, Sánchez-Iglesias S, Araújo-Vilar D, Fong LG, Young SG.

Nucleus. 2016 Sep 2;7(5):512-521.


Mobility of "HSPG-bound" LPL explains how LPL is able to reach GPIHBP1 on capillaries.

Allan CM, Larsson M, Jung RS, Ploug M, Bensadoun A, Beigneux AP, Fong LG, Young SG.

J Lipid Res. 2017 Jan;58(1):216-225. doi: 10.1194/jlr.M072520. Epub 2016 Nov 3.


NanoSIMS imaging: an approach for visualizing and quantifying lipids in cells and tissues.

He C, Fong LG, Young SG, Jiang H.

J Investig Med. 2017 Mar;65(3):669-672. doi: 10.1136/jim-2016-000239. Epub 2016 Oct 28. Review.


An LPL-specific monoclonal antibody, 88B8, that abolishes the binding of LPL to GPIHBP1.

Allan CM, Larsson M, Hu X, He C, Jung RS, Mapar A, Voss C, Miyashita K, Machida T, Murakami M, Nakajima K, Bensadoun A, Ploug M, Fong LG, Young SG, Beigneux AP.

J Lipid Res. 2016 Oct;57(10):1889-1898. Epub 2016 Aug 5.


GPIHBP1 and Plasma Triglyceride Metabolism.

Fong LG, Young SG, Beigneux AP, Bensadoun A, Oberer M, Jiang H, Ploug M.

Trends Endocrinol Metab. 2016 Jul;27(7):455-469. doi: 10.1016/j.tem.2016.04.013. Epub 2016 May 14. Review.


Lamin B1 and lamin B2 are long-lived proteins with distinct functions in retinal development.

Razafsky D, Ward C, Potter C, Zhu W, Xue Y, Kefalov VJ, Fong LG, Young SG, Hodzic D.

Mol Biol Cell. 2016 Jun 15;27(12):1928-37. doi: 10.1091/mbc.E16-03-0143. Epub 2016 Apr 13.


Modulation of LMNA splicing as a strategy to treat prelamin A diseases.

Lee JM, Nobumori C, Tu Y, Choi C, Yang SH, Jung HJ, Vickers TA, Rigo F, Bennett CF, Young SG, Fong LG.

J Clin Invest. 2016 Apr 1;126(4):1592-602. doi: 10.1172/JCI85908. Epub 2016 Mar 21.


Palmoplantar Keratoderma in Slurp2-Deficient Mice.

Allan CM, Procaccia S, Tran D, Tu Y, Barnes RH 2nd, Larsson M, Allan BB, Young LC, Hong C, Tontonoz P, Fong LG, Young SG, Beigneux AP.

J Invest Dermatol. 2016 Feb;136(2):436-443. doi: 10.1016/j.jid.2015.11.003. Epub 2015 Nov 18.


The acidic domain of the endothelial membrane protein GPIHBP1 stabilizes lipoprotein lipase activity by preventing unfolding of its catalytic domain.

Mysling S, Kristensen KK, Larsson M, Beigneux AP, Gårdsvoll H, Fong LG, Bensadouen A, Jørgensen TJ, Young SG, Ploug M.

Elife. 2016 Jan 3;5:e12095. doi: 10.7554/eLife.12095.


SREBP-2-deficient and hypomorphic mice reveal roles for SREBP-2 in embryonic development and SREBP-1c expression.

Vergnes L, Chin RG, de Aguiar Vallim T, Fong LG, Osborne TF, Young SG, Reue K.

J Lipid Res. 2016 Mar;57(3):410-21. doi: 10.1194/jlr.M064022. Epub 2015 Dec 18.


Heterogeneity in the properties of mutant secreted lymphocyte antigen 6/urokinase receptor-related protein 1 (SLURP1) in Mal de Meleda.

Adeyo O, Oberer M, Ploug M, Fong LG, Young SG, Beigneux AP.

Br J Dermatol. 2015 Oct;173(4):1066-9. doi: 10.1111/bjd.13868. Epub 2015 Aug 19. No abstract available.


Nuclear envelope protein Lem2 is required for mouse development and regulates MAP and AKT kinases.

Tapia O, Fong LG, Huber MD, Young SG, Gerace L.

PLoS One. 2015 Mar 19;10(3):e0116196. doi: 10.1371/journal.pone.0116196. eCollection 2015.


Mice that express farnesylated versions of prelamin A in neurons develop achalasia.

Yang SH, Procaccia S, Jung HJ, Nobumori C, Tatar A, Tu Y, Bayguinov YR, Hwang SJ, Tran D, Ward SM, Fong LG, Young SG.

Hum Mol Genet. 2015 May 15;24(10):2826-40. doi: 10.1093/hmg/ddv043. Epub 2015 Feb 4.


Do lamin B1 and lamin B2 have redundant functions?

Lee JM, Jung HJ, Fong LG, Young SG.

Nucleus. 2014 Jul-Aug;5(4):287-92. doi: 10.4161/nucl.29615.


GPIHBP1 missense mutations often cause multimerization of GPIHBP1 and thereby prevent lipoprotein lipase binding.

Beigneux AP, Fong LG, Bensadoun A, Davies BS, Oberer M, Gårdsvoll H, Ploug M, Young SG.

Circ Res. 2015 Feb 13;116(4):624-32. doi: 10.1161/CIRCRESAHA.116.305085. Epub 2014 Nov 11.


Sphingosine-1-phosphate lyase downregulation promotes colon carcinogenesis through STAT3-activated microRNAs.

Degagné E, Pandurangan A, Bandhuvula P, Kumar A, Eltanawy A, Zhang M, Yoshinaga Y, Nefedov M, de Jong PJ, Fong LG, Young SG, Bittman R, Ahmedi Y, Saba JD.

J Clin Invest. 2014 Dec;124(12):5368-84. doi: 10.1172/JCI74188. Epub 2014 Oct 27.


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.

Jung HJ, Tatar A, Tu Y, Nobumori C, Yang SH, Goulbourne CN, Herrmann H, Fong LG, Young SG.

Mol Cell Biol. 2014 Dec;34(24):4534-44. doi: 10.1128/MCB.00997-14. Epub 2014 Oct 13.


High-resolution imaging of dietary lipids in cells and tissues by NanoSIMS analysis.

Jiang H, Goulbourne CN, Tatar A, Turlo K, Wu D, Beigneux AP, Grovenor CR, Fong LG, Young SG.

J Lipid Res. 2014 Oct;55(10):2156-66. doi: 10.1194/jlr.M053363. Epub 2014 Aug 20.


Multimerization of glycosylphosphatidylinositol-anchored high density lipoprotein-binding protein 1 (GPIHBP1) and familial chylomicronemia from a serine-to-cysteine substitution in GPIHBP1 Ly6 domain.

Plengpanich W, Young SG, Khovidhunkit W, Bensadoun A, Karnman H, Ploug M, Gårdsvoll H, Leung CS, Adeyo O, Larsson M, Muanpetch S, Charoen S, Fong LG, Niramitmahapanya S, Beigneux AP.

J Biol Chem. 2014 Jul 11;289(28):19491-9. doi: 10.1074/jbc.M114.558528. Epub 2014 May 20.


Nuclear lamins and neurobiology.

Young SG, Jung HJ, Lee JM, Fong LG.

Mol Cell Biol. 2014 Aug;34(15):2776-85. doi: 10.1128/MCB.00486-14. Epub 2014 May 19. Review.


The GPIHBP1-LPL complex is responsible for the margination of triglyceride-rich lipoproteins in capillaries.

Goulbourne CN, Gin P, Tatar A, Nobumori C, Hoenger A, Jiang H, Grovenor CR, Adeyo O, Esko JD, Goldberg IJ, Reue K, Tontonoz P, Bensadoun A, Beigneux AP, Young SG, Fong LG.

Cell Metab. 2014 May 6;19(5):849-60. doi: 10.1016/j.cmet.2014.01.017. Epub 2014 Apr 10.


Equivalent binding of wild-type lipoprotein lipase (LPL) and S447X-LPL to GPIHBP1, the endothelial cell LPL transporter.

Turlo K, Leung CS, Seo JJ, Goulbourne CN, Adeyo O, Gin P, Voss C, Bensadoun A, Fong LG, Young SG, Beigneux AP.

Biochim Biophys Acta. 2014 Jul;1841(7):963-9. doi: 10.1016/j.bbalip.2014.03.011. Epub 2014 Apr 2.


A new monoclonal antibody, 4-1a, that binds to the amino terminus of human lipoprotein lipase.

Bensadoun A, Mottler CD, Pelletier C, Wu D, Seo JJ, Leung CS, Adeyo O, Goulbourne CN, Gin P, Fong LG, Young SG, Beigneux AP.

Biochim Biophys Acta. 2014 Jul;1841(7):970-6. doi: 10.1016/j.bbalip.2014.03.008. Epub 2014 Mar 28.


Reciprocal knock-in mice to investigate the functional redundancy of lamin B1 and lamin B2.

Lee JM, Tu Y, Tatar A, Wu D, Nobumori C, Jung HJ, Yoshinaga Y, Coffinier C, de Jong PJ, Fong LG, Young SG.

Mol Biol Cell. 2014 May;25(10):1666-75. doi: 10.1091/mbc.E14-01-0683. Epub 2014 Mar 26.


Stable isotope imaging of biological samples with high resolution secondary ion mass spectrometry and complementary techniques.

Jiang H, Favaro E, Goulbourne CN, Rakowska PD, Hughes GM, Ryadnov MG, Fong LG, Young SG, Ferguson DJ, Harris AL, Grovenor CR.

Methods. 2014 Jul 1;68(2):317-24. doi: 10.1016/j.ymeth.2014.02.012. Epub 2014 Feb 17.

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