Shriners Research Center, Portland OR

Horton Laboratory Bächinger Laboratory Sakai Laboratory Hurlin Laboratory Stadler Laboratory Schweitzer Laboratory Keene Laboratory

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., .Epub, 2005, June 1

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.

Carta L, Arteaga-Solis E, Lee-Arteaga SY, Lenart B, Starcher B, Merkel CA, Sukoyan M, Kerkis A, Hazeki N, Keene DR, Sakai LY, Ramirez F, Fibrillins 1 and 2 perform partially overlapping functions during aortic development. J Biol Chem. 2005 Dec 28; [Epub ahead of print] PMID: 16407178 [PubMed - as supplied publisher]

Brinckmann J, Hunzelmann N, El-Hallous E, Krieg T, Sakai LY, Krengel S, Reinhardt DP. Absence of autoantibodies against correctly folded recombinant fibrillin-1 protein in systemic sclerosis patients. Arthritis Res Ther. 2005;7(6):R1221-6. Epub 2005 Sept 6.PMID:16277674 [PubMed - indexed for MEDLINE]

Kadoya K, Sasaki T, Kostka G, Timpl R, Matsuzaki K, Kumagai N, Sakai LY, Nishiyama T, Amano S, Fibulin-5 deposition in human skin: decrease with ageing and ultraviolet B exposure and increase in solar elastosis. Dr J Dermatol. 2005 Sep; 153(3):607-12. PMID:16120151 [PUbMed - indexed for MEDLINE]

Jordan CD, Charbonneau NL, Sakai LY, Fibrillin microfibrils: Connective tissue pathways that regulate shape and signalling., J Muscoloskelet Neuronal Interact. 2006 Oct-Dec;6(4):366-7.

Kuo CL, Isogai Z, Keene DR, Hazeki N, Ono RN, Sengle G, Bachinger HP, Sakai LY, Effects of fibrillin-1 degradation on microfibril ultrastructure. J Biol Chem. 2007 Feb9;282(6):4007-20. Epub 2006 Dec 7. PMID:17158461 [PubMed - indexed for MEDLINE]

Vainionpaa N, Butzow R, Hukkanen M, Jackson DG, Phihlajaniemi T, Sakai LY, Virtanen I, Basement membrane protein distribution in LYVE-1-immunoreactive lymphatic vessels of normal tissues and ovarian carcinomas. Cell Tissue Res. 2007 May;328(2):317-28. Epub 2007 Jan 30. PMID: 17265066 [PubMed - indexed for MEDLINE]

Ramirez F, Sakai LY, Rifkin DB, Dietz HC, Extracellular microfibrils in development and disease. Cell Mol Life Sci. 2007 Sep;64(18):2437-46. PMID:17585369 [PubMed indexed for MEDLINE]

Zilberberg L, Ten Dijke P, Sakai LY, Rifkin DB, A rapid and sensitive bioassay to measure bone morphogenetic protein activity. BMC Cell Biol. 2007 Sep19;8(1):41 [Epub ahead of print] PMID: 17880711 [PUbMed - as supplied by publisher]

Dasouki M, Karkova D, Garola R, Sasaki T, Charbonneau NL, Sakai LY, Chu ML, Compund heterozygous mutations in fibulin-4 causing neonatal lethal pulmonary artery occlusion, aortic aneurysm, arachnodactyly, and mild cutis laxa. Am J. Med Genet A. 2007 Nov 15;143(22):2635-41. PMID:17397443 [PubMed - in process]

Sengle G, Charbonneau NL, Ono RN, Sasaki T, Alvarez J, Keene DR, Bachinger HP, Sakai LY Targetting of bone morphogenetic protein growth factor complexes to fibrillin. J Biol Chem. 2008 Mar 13, [Epub ahead of print]

Ohno-Jimo A, Isogai Z, Yoneda M, Kasai K, Miyaishi O, Inoue Y, Kataoka T, Zhao JS, Li H, Takeyama M, Keene DR, Sakai LY, Kimata K, Iwaki M, Zako M, Versican and Fibrillin-1 form a major hyaluronan-binding complex in the ciliary body, Invest Ophthalmol Vis Sci. 2008 Apr 4; [Epub ahead of print] PMID:18390636 [PUbMed - as supplied by publisher]

Fricke M, Langer C, Brunner E, Sakai LY, Fuzesi L, Reinhardt DP, Quondamatteo F, Fribrillin-1 in incisional hernias: an immunohistochemical study in scar and non-scar regions of human skin and muscle fasciae, J Anat. 2008 A[ro; 10; [Epub ahead of print] PMID:18410314 [PubMed - as supplied by publisher]