Hui Sun, PhD

Position Title:
Professor, Department of Physiology
Office: 53-140 CHS
Phone: 310- 206-4017
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Hui Sun, Ph.D. Bibliography

    The 7 papers that have been cited for more than 300 times are marked by•••

  1. Allikmets, R.; Singh, N.; Sun, H.; Shroyer, N. F.; Hutchinson, A.; Chidambaram, A.; Gerrard, B.; Baird, L.; Stauffer, D.; Peiffer, A.; Rattner, A.; Smallwood, P.; Li, Y.; Anderson, K. L.; Lewis, R. A.; Nathans, J.; Leppert, M.; Dean, M.; Lupski, J. R., A photoreceptor cell-specific ATP-binding transporter gene (ABCR) is mutated in recessive Stargardt macular dystrophy. Nature Genetics 1997, 15 (3), 236-46.•••
  2. Sun, H.; Macke, J. P.; Nathans, J., Mechanisms of spectral tuning in the mouse green cone pigment. Proc Natl Acad Sci U S A 1997, 94 (16), 8860-5.
  3. Chen, S.; Wang, Q. L.; Nie, Z.; Sun, H.; Lennon, G.; Copeland, N. G.; Gilbert, D. J.; Jenkins, N. A.; Zack, D. J., Crx, a novel Otx-like paired-homeodomain protein, binds to and transactivates photoreceptor cell-specific genes. Neuron 1997, 19 (5), 1017-30.•••
  4. Sun, H.; Gilbert, D. J.; Copeland, N. G.; Jenkins, N. A.; Nathans, J., Peropsin, a novel visual pigment-like protein located in the apical microvilli of the retinal pigment epithelium. Proc Natl Acad Sci U S A 1997, 94 (18), 9893-8.
  5. Sun, H.; Nathans, J., Stargardt's ABCR is localized to the disc membrane of retinal rod outer segments. Nature Genetics 1997, 17 (1), 15-6.
  6. Sherman, P. M.; Sun, H.; Macke, J. P.; Williams, J.; Smallwood, P. M.; Nathans, J., Identification and characterization of a conserved family of protein serine/threonine phosphatases homologous to Drosophila retinal degeneration C. Proc Natl Acad Sci U S A 1997, 94 (21), 11639-44.
  7. Sun, H.; Molday, R. S.; Nathans, J., Retinal stimulates ATP hydrolysis by purified and reconstituted ABCR, the photoreceptor-specific ATP-binding cassette transporter responsible for Stargardt disease. J Biol Chem 1999, 274 (12), 8269-81.•••
  8. Rattner, A.; Sun, H.; Nathans, J., Molecular genetics of human retinal disease. Annu Rev Genet 1999, 33, 89-131.
  9. Xu, S.; Ladak, R.; Swanson, D. A.; Soltyk, A.; Sun, H.; Ploder, L.; Vidgen, D.; Duncan, A. M.; Garami, E.; Valle, D.; McInnes, R. R., PHR1 encodes an abundant, pleckstrin homology domain-containing integral membrane protein in the photoreceptor outer segments. J Biol Chem 1999, 274 (50), 35676-85.
  10. Sun, H.; Smallwood, P. M.; Nathans, J., Biochemical defects in ABCR protein variants associated with human retinopathies. Nature Genetics 2000, 26 (2), 242-6.
  11. Sun, H.; Nathans, J., ABCR: rod photoreceptor-specific ABC transporter responsible for Stargardt disease. Methods Enzymol 2000, 315, 879-97.
  12. Sun, H.; Nathans, J., Mechanistic studies of ABCR, the ABC transporter in photoreceptor outer segments responsible for autosomal recessive Stargardt disease. J Bioenerg Biomembr 2001, 33 (6), 523-30.
  13. Sun, H.; Nathans, J., ABCR, the ATP-binding cassette transporter responsible for Stargardt macular dystrophy, is an efficient target of all-trans-retinal-mediated photooxidative damage in vitro. Implications for retinal disease. J Biol Chem 2001, 276 (15), 11766-74.
  14. Sun, H.; Nathans, J., The challenge of macular degeneration. Scientific American 2001, 285 (4), 68-75.
  15. Sun, H.; Tsunenari, T.; Yau, K. W.; Nathans, J., The vitelliform macular dystrophy protein defines a new family of chloride channels. Proc Natl Acad Sci U S A 2002, 99 (6), 4008-13.•••

    This paper discovered a new family of anion channels in vertebrate and invertebrate animals and was highlighted in the following scientific news:
    Featured in the Howard Hughes Medical Institute News
    Rated as Must ReadbyFaculty of 1000 Biology
    This anion channel family participates in diverse physiological functions such as tonic GABA release from glia (Science 330:790-6, 2010) and sweat production (PNAS 109: 1199-203,2012).
    The crystal structures of this new type of anion channels have been determined (Science 346:355-9, 2014 and Nature 516: 213-8, 2014)

  16. Tsunenari, T.; Sun, H.; Williams, J.; Cahill, H.; Smallwood, P.; Yau, K. W.; Nathans, J., Structure-function analysis of the bestrophin family of anion channels. J Biol Chem 2003, 278 (42), 41114-25.
  17. Jin, M.; Li, S.; Moghrabi, W. N.; Sun, H.; Travis, G. H., RPE65 is the retinoid isomerase in bovine retinal pigment epithelium. Cell 2005, 122 (3), 449-59.•••
  18. Yang, Z.; Camp, N. J.; Sun, H.; Tong, Z.; Gibbs, D.; Cameron, D. J.; Chen, H.; Zhao, Y.; Pearson, E.; Li, X.; Chien, J.; Dewan, A.; Harmon, J.; Bernstein, P. S.; Shridhar, V.; Zabriskie, N. A.; Hoh, J.; Howes, K.; Zhang, K., A variant of the HTRA1 gene increases susceptibility to age-related macular degeneration. Science2006, 314 (5801), 992-3.•••
  19. Kawaguchi, R.; Yu, J.; Honda, J.; Hu, J.; Whitelegge, J.; Ping, P.; Wiita, P.; Bok, D.; Sun, H., A membrane receptor for retinol binding protein mediates cellular uptake of vitamin A. Science (Research Article)2007, 315 (5813), 820-5.•••

  20. This article was highlighted in the following scientific news:
    Science News, Science January 25, 2007
    Research Highlights, Nature 445: 462, 2007
    Editors Choice, Science STKE 373:55, 2007
    Research Spotlight, ACS Chemical Biology 2:142, 2007
    This week in Science, Science 315: 735, 2007
    Research News, The Scientist, January 25, 2007
    Rated as Exceptional by Faculty of 1000 Biology

  21. Noble, B. R.; Babiuk, R. P.; Clugston, R. D.; Underhill, T. M.; Sun, H.; Kawaguchi, R.; Walfish, P. G.; Blomhoff, R.; Gundersen, T. E.; Greer, J. J., Mechanisms of action of the congenital diaphragmatic hernia-inducing teratogen nitrofen. Am J Physiol 2007, 293 (4), L1079-87.
  22. Yu, J.; Wiita, P.; Kawaguchi, R.; Honda, J.; Jorgensen, A.; Zhang, K.; Fischetti, V. A.; Sun, H., Biochemical analysis of a common human polymorphism associated with age-related macular degeneration. Biochemistry 2007, 46 (28), 8451-61.
  23. Kawaguchi, R.; Yu, J.; Wiita, P.; Ter-Stepanian, M.; Sun, H., Mapping the membrane topology and extracellular ligand binding domains of the retinol binding protein receptor. Biochemistry 2008, 47 (19), 5387-95.
  24. Kawaguchi, R.; Yu, J.; Wiita, P.; Honda, J.; Sun, H., An essential ligand-binding domain in the membrane receptor for retinol-binding protein revealed by large-scale mutagenesis and a human polymorphism. J Biol Chem 2008, 283 (22), 15160-8.
  25. Pavone, M. E.; Reierstad, S.; Sun, H.; Milad, M.; Bulun, S. E.; Cheng, Y. H., Altered retinoid uptake and action contributes to cell survival in endometriosis. J Clin Endocrinol Metab 2010, 95 (11), E300-9.
  26. Ufret-Vincenty, R. L.; Aredo, B.; Liu, X.; McMahon, A.; Chen, P. W.; Sun, H.; Niederkorn, J. Y.; Kedzierski, W., Transgenic mice expressing variants of complement factor H develop AMD-like retinal findings. Invest Ophthalmol Vis Sci 2010, 51 (11), 5878-87.
  27. Kawaguchi, R.; Sun, H., Techniques to study specific cell-surface receptor-mediated cellular vitamin A uptake. Methods Mol Biol 2010, 652, 341-61.
  28. Kawaguchi, R.; Yu, J.; Ter-Stepanian, M.; Zhong, M.; Cheng, G.; Yuan, Q.; Jin, M.; Travis, G. H.; Ong, D.; Sun, H., Receptor-mediated cellular uptake mechanism that couples to intracellular storage. ACS Chemical Biology 2011, 6 (10), 1041-51.
  29. Sun, H.; Kawaguchi, R., The membrane receptor for plasma retinol-binding protein, a new type of cell-surface receptor. Int Rev Cell Mol Biol 2011, 288, 1-41.
  30. Casey, J.; Kawaguchi, R.; Morrissey, M.; Sun, H.; McGettigan, P.; Nielsen, J. E.; Conroy, J.; Regan, R.; Kenny, E.; Cormican, P.; Morris, D. W.; Tormey, P.; Chroinin, M. N.; Kennedy, B. N.; Lynch, S.; Green, A.; Ennis, S., First implication of STRA6 mutations in isolated anophthalmia, microphthalmia, and coloboma: a new dimension to the STRA6 phenotype. Hum Mutat 2012, 32 (12), 1417-26.
  31. Kawaguchi, R.; Zhong, M.; Kassai, M.; Ter-Stepanian, M.; Sun, H., STRA6-catalyzed vitamin A influx, efflux and exchange. J Membr Biol. 2012, 245 (11), 731-745.
  32. Sun, H., Membrane receptors and transporters involved in the function and transport of vitamin A and its derivatives. Biochim Biophys Acta 2012, 1821 (1), 99-112.
  33. Shaw, P. X.; Zhang, L.; Zhang, M.; Du, H.; Zhao, L.; Lee, C.; Grob, S.; Lim, S. L.; Hughes, G.; Lee, J.; Bedell, M.; Nelson, M. H.; Lu, F.; Krupa, M.; Luo, J.; Ouyang, H.; Tu, Z.; Su, Z.; Zhu, J.; Wei, X.; Feng, Z.; Duan, Y.; Yang, Z.; Ferreyra, H.; Bartsch, D. U.; Kozak, I.; Lin, F.; Sun, H.; Feng, H.; Zhang, K., Complement factor H genotypes impact risk of age-related macular degeneration by interaction with oxidized phospholipids. Proc Natl Acad Sci U S A 2012, 109 (34), 13757-62.
  34. Zhong, M.; Kawaguchi, R.; Kassai, M.; Sun, H., Retina, retinol, retinal and the natural history of vitamin A as a light sensor. Nutrients 2012, 4 (12), 2069-96.
  35. Kawaguchi, R.; Zhong, M.; Sun, H., Real-time analyses of retinol transport by the membrane receptor of plasma retinol binding protein. J Vis Exp 2013, (71), e50169.
  36. Kawaguchi, R.; Zhong, M.; Kassai, M.; Ter-Stepanian, M.; Sun, H., Differential and isomer-specific modulation of vitamin A transport and the catalytic activities of the RBP receptor by retinoids. J Membr Biol 2013, 246 (8), 647-60.
  37. Zhong, M.; Kawaguchi, R.; Ter-Stepanian, M.; Kassai, M.; Sun, H., Vitamin A transport and the transmembrane pore in the cell-surface receptor for plasma retinol binding protein. PLoS One 2013, 8 (11), e73838.
  38. Terra, R.; Wang, X.; Hu, Y.; Charpentier, T.; Lamarre, A.; Zhong, M.; Sun, H.; Mao, J.; Qi, S.; Luo, H.; Wu, J., To Investigate the Necessity of STRA6 Upregulation in T Cells during T Cell Immune Responses. PLoS One 2013, 8 (12), e82808.< /li>
  39. Zhong, M.; Kawaguchi, R.; Kassai, M.; Sun, H., Apo-RBP, holo-RBP, and insulin resistance. Mol Cell Biol 2014, 34 (11), 2105-6.
  40. Zhong, M.; Kawaguchi, R.; Kassai, M.; Sun, H., How free retinol behaves differently from rbp-bound retinol in RBP receptor-mediated vitamin A uptake. Mol Cell Biol 2014, 34 (11), 2108-10.
  41. Esmailpour, T.; Riazifar, H.; Liu, L.; Donkervoort, S.; Huang, V. H.; Madaan, S.; Shoucri, B. M.; Busch, A.; Wu, J.; Towbin, A.; Chadwick, R. B.; Sequeira, A.; Vawter, M. P.; Sun, G.; Johnston, J. J.; Biesecker, L. G.; Kawaguchi, R.; Sun, H.; Kimonis, V.; Huang, T., A splice donor mutation in NAA10 results in the dysregulation of the retinoic acid signalling pathway and causes Lenz microphthalmia syndrome. J Med Genet 2014, 51 (3), 185-96.
  42. Cheng, G.*; Zhong, M.*; Kawaguchi, R.*; Kassai, M.; Al-Ubaidi, M.; Deng, J.; Ter-Stepanian, M.; Sun, H., Identification of PLXDC1 and PLXDC2 as the transmembrane receptors for the multifunctional factor PEDF. eLife 2014, 4 (doi: 10.7554/eLife.05401). *These authors contributed equally to this work.
  43. Zhong, M; Sun, H., A Genetic Clog in the Vitamin A Transport Machinery. Cell 2015, 161 (3), 435-437.