Selected Recent Publications
Original articles
Vranka, J.; Mokashi, A.; Keene, D.R.; Tufa, S.; Corson, G.; Sussman, M.; Horton, W.A.; Maddox, K.; Sakai, L.; and Bachinger, H.P. Selective intracellular retention of extracellular matrix proteins and chaperones associated with pseudoachondroplasia. Matrix Biol, 20(7):439-50, 2001.
Arteaga-Solis, E., Gayraud, B., Lee, S.Y., Shum, L., Sakai, L., and Ramirez, F.: Regulation of limb patterning by extracellular microfibrils. J. Cell Biol., 154:275-281, 2001.
Dzamba, B.J.; Keene, D.R.; Isogai, Z.; Charbonneau, N.L.; Karaman-Jurukovska, N.; Simon, M.; and Sakai, L.Y. Assembly of epithelial cell fibrillins. J Invest Dermatol, 117(6):1612-20, 2001.
Schlotzer-Schrehardt, U.; Zenkel, M.; Kuchle, M.; Sakai, L.Y.; and Naumann, G.O. Role of transforming growth factor-beta1 and its latent form binding protein in pseudoexfoliation syndrome. Exp Eye Res, 73(6):765-80, 2001.
Sakai , L.Y.; Byers, P.H.; and Ramirez, F. A report on the 3rd Workshop on Heritable Disorders of Connective Tissue. Matrix Biol, 21(1):7-13, 2001.
Quondamatteo, F.; Reinhardt, D.P.; Charbonneau, N.L.; Pophal, G.; Sakai, L.Y.; and Herken, R. Fibrillin-1 and fibrillin-2 in human embryonic and early fetal development. Matrix Biol, 21(8):637-46, 2002.
Isogai, Z.; Aspberg, A.; Keene, D.R.; Ono, R.N.; Reinhardt, D.P.; and Sakai, L.Y. Versican interacts with fibrillin-1 and links extracellular microfibrils to other connective tissue networks. J Biol Chem, 277(6):4565-72, 2002.
Neptune, E.R.; Frischmeyer, P.A.; Arking, D.E.; Myers, L.; Bunton, T.E.; Gayraud, B.; Ramirez, F.; Sakai, L.Y.; and Dietz, H.C. Dysregulation of TGF-beta activation contributes to pathogenesis in Marfan syndrome. Nat Genet, 33(3):407-11, 2003.
Samuel, C.S.; Sakai, L.Y.; and Amento, E.P. Relaxin regulates fibrillin 2, but not fibrillin 1, mRNA and protein expression by human dermal fibroblasts and murine fetal skin. Arch Biochem Biophys, 411(1):47-55, 2003.
Charbonneau, N.L.; Dzamba, B.J.; Ono, R.N.; Keene, D.R.; Corson, G.M.; Reinhardt, D.P.; and Sakai, L.Y. Fibrillins can co-assemble in fibrils, but fibrillin fibril composition displays cell-specific differences. J Biol Chem, 278(4):2740-9, 2003.
Isogai, Z.; Ono, R.N.; Ushiro, S.; Keene, D.R.; Chen, Y.; Mazzieri, R.; Charbonneau, N.L.; Reinhardt, D.P.; Rifkin, D.B.; and Sakai, L.Y. Latent transforming growth factor beta-binding protein 1 interacts with fibrillin and is a microfibril-associated protein. J Biol Chem, 278(4):2750-7, 2003.
Isogai, Z., Gregory, K.E., Ono, R.N., Charbonneau, N.L., Corson, G.M., Keene, D.R., and Sakai, L.Y.: Microfibrils and morphogenesis. In Elastin 2002 (A.M. Tamburro and A. Pepe, eds.), Editric Ermes, Potenza, Italy, pp. 213-223, 2003.
Corson, G.M.; Charbonneau, N.L.; Keene, D.R.; and Sakai, L.Y. Differential expression of fibrillin-3 adds to microfibril variety in human and avian, but not rodent, connective tissues. Genomics, 83(3):461-72, 2004.
Vranka, J.A., Sakai, L.Y., and Bächinger, H.P.: Prolyl 3-hydroxylase 1, enzyme characterization and identification of a novel family of enzymes. J Biol Chem , 279:23615-23621, 2004
Sakai , L.Y.: The fibrillins and key molecular mechanisms that initiate disease pathways. In The Marfan Syndrome (P. Robinson and M. Godfrey, eds), Landes, 178-187, 2004.
Charbonneau, N.L., Ono, R.N., Corson, G.M., Keene, D.R., and Sakai, L.Y.: Fine tuning of growth factor signals depends on fibrillin microfibril networks. Birth Defects Research (Part C: Embryo Today), 72:37-50 , 2004.
Judge, D.P., Biery, N.J., Keene, D.R., Geubtne, J., Myers, L., Huso, D.L., Sakai, L.Y., Dietz, H.C.: Evidence for a critical contribution of haploinsufficiency in the complex pathogenesis of Marfan syndrome. J Clin Invest . 114:172-181, 2004.
Ramirez, F., Sakai, L.Y., Dietz, H.C., Rifkin, D.B.: Fibrillin microfibrils: multipurpose extracellular networks in organismal physiology. Physiol Genomics 19:151-154, 2004.
Ortiz-Urda S, Garcia J, Green CL, Chen L, Lin Q, Veitch DP, Sakai LY, Lee H, Marinkovich MP, Khavari PA.:Type VII collagen is required for Ras-driven human epidermal tumorigenesis. Science 307:1773-1776, 2005.
Gregory, K.E., Ono, R.N., Charbonneau, N.L., Kuo, C-L., Keene, D.R., Bächinger, H.P., and Sakai, L.Y. The prodomain of BMP-7 targets the BMP-7 complex to the extracellular matrix. J. Biol. Chem., in press, 2005.
Fontana , L., Chen, Y., Prijatelj, P., Sakai, T., Fässler, R., Sakai, L.Y., and Rifkin, D.B. Fibronectin is required for a v b 6-mediated activation of latent TGF b . FASEB J, in revision, 2005.
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