Shriners Research Center, Portland OR

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William Horton | Hans Peter Bächinger | Lynn Sakai | Peter Hurlin | Scott Stadler | Ronen Schweitzer | Douglas Keene
6th Pan Pacific Connective Tissue Symposium | Skeletal Growth Workshop | Seminar Series

Publications

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One of the most traditional aspects of research, and one of the most critical, is the publication of scientific findings and information through peer reviewed journal articles, books, book chapters, and more. Every year, our Principal Investigators publish scores of articles and other scientific material as they continuously pursue further advancements in their respective fields.

Below you will find the latest publications produced by our researchers, as well as a list of publications from the past year. Please feel free to follow the links and find out more about our current research interests and programs.


Publications in 2007
Horton WA, Hall JG, Hecht JT: Achondroplasia Lancet. 2007 Jul 14;370(9582):162-72. Review PMID: 17630040 [PubMed - indexed for MEDLINE]

Horton WA, Tuan RS, Jacenko O. International workshop on the Skeletal Growth Plate Stevenson, Washington, June 11-15, 2006. Matrix Biol. 2007 Feb 20; No abstract available. PMID: 17391950

Alman BA, Horton WA Developmental biology in orthopaedics. Summary of the 2006 AAOS research symposium. J Bone Joint Surg Am. 2007 Mar;89(3):668-71. No abstract available. PMID: 17332117

NG PY, Tang Y, Knosp WM, Stadler HS, Shaw JT Synthesis of Diverse Lactam Carboxamides Leading to the Discovery of a New Transcription-Factor Inhibitor. Angew Chem Int Ed Engl. 2007 Jul 9;46(28):5352-5355. No abstract available. PMID: 17568465

Shaut CA, Saneyoshi C, Morgan EA, Knosp WM, Sexton DR, Stadler HS. HOXA13 directly regulates EphA6 and EphA7 expression in the genital tubercle vascular endothelia. Dev Dyn. 2007 Apr:236(4):951-60. PMID: 17304517

Akiyama H, Stadler HS, Martin JF, Ishii TM, Beachy PA, Nakamura T, de Crombrugghe B. Misexpression of Sox9 in mouse limb bud mesenchyme induces polydactyly and rescues hypodactyly mice. Matrix Biol. 2007 May 26(4):224-33. Epub 2006 Dec 8. PMID 17222543

Knosp WM, Saneyoshi C, Shou S, Bachinger HP, Stadler HS. Elucidation, quantitative refinement, and in vivo utilization of the HOXA13 DNA binding site. J Biol Chem. 2007 Mar 2;282(9):6843-53. Epub 2007 Jan 1. PMID: 17200107

Meier S, Jensen PR, Adamczyk P, Bachinger HP, Holstin RW, Engel J, Ozbek S, Grzesiek S. Sequence-Structure and Structure-Function Analysis in Cysteine-rich Domains Forming the Ultrastable Nematocyst Wall. J Mol Biol. 2007 May 4;368(3):718-28. Epub 2007 Feb-24 PMID: 17362991

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

Vainionpaa N, Butzow R, Hukkanen M, Jackson DG, Pihlajaniemi 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

Murchison ND, Pryce BA, Conner DA, Keene DR, Olson EN, Tabin CJ, Schweitzer R. Regulation of tendon differentiation by scleraxis distinguishes force-transmitting tendons from muscle-anchoring tendons. Development. 2007 Jul;134(14):2697-708. Epub 2007 Jun 13 PMID: 17567668

Russell BH, Vasan R, Keene DR, Xu Y. Bacillus anthracis internalization by human fibroblasts and epithelial cells. Cell Microbiol. 2007 May;9(5):1262-74. PMID: 17474904

Ota, S., Zhou, ZQ., Keene, DR., Knoepfler, P., Hurlin, PJ Activities of N-Myc in the developing limb link control of skeletal size and digit separation. Development. 2007 Apr;134(8):1583-92. Epub 2007 Mar 14. PMID: 17360777

Kobayashi N, Kostka G, Garbe JH, Keene DR, Bachinger HP, Hanisch FG, Markova D, Tsuda T, Timpl R, Chu ML, Sasaki T. A comparative analysis of the fibulin protein family: Biochemical characterization, binding interactions and tissue localization. J Biol Chem. 2007 Apr 20;282(16):11805-16. Epub 2007 Feb-26; PMID: 17324935

Guarnieri D, Battista S, Borzacchiello A, Mayol L, De Rosa E, Keene DR, Muscariello L, Barbarisi A, A Netti P. Effects of fibronectin and laminin on structural, mechanical and transport properties of 3D collageneous network. J Mater Sci Mater Med. 2007 Feb;18(2):245-53. PMID: 17323155

Weirich C, Keene DR, Kirsch K, Heil M, Neumann E, Dinser R. Expression of PSACH-associated mutant COMP in tendon fibroblasts leads to increased apoptotic cell death irrespective of the secretory characteristics of mutant COMP. Matrix Biol. May:26(4):314-323. Epub 2007 Jan 18; PMID: 17307347

Maertens B, Hopkins D, Franzke CW, Keene DR, Bruckner-Tuderman L, Greenspan DS, Koch M. Cleavage and oligomerization of gliomedin, a transmembrane collagen required for node of Ranvier formation. J Biol Chem. Apr;282(14):10647-59. Epub 2007 Feb 8 PMID: 17293346

Woodley DT, Remington J, Huang Y, Hou Y, Li W, Keene DR, Chen M. Intravenously Injected Human Fibroblasts Home to Skin Wounds, Deliver Type VII Collagen, and Promote Wound Healing. Mol Ther. 2007 Mar;15(3):628-35. Epub 2007 Jan 23. PMID: 17245357

Cabral WA, Makareeva E, Letocha AD, Scribanu N, Fertala A, Steplewski A, Keene DR, Persikov AV, Leikin S, Marini JC. Y-position cysteine substitution in type I collagen (alpha1(I) R888C/p.R1066C) is associated with osteogenesis imperfecta/Ehlers-Danlos syndrome phenotype. Hum Mutat. 2007 Apr;28(4):396-405. Epub 2007 Jan 5. PMID: 17206620

Sonnylal S, Denton CP, Zheng B, Keene DR, He R, Adams HP, Vanpelt CS, Geng Yj, Deng JM, Behringer RR, de Crombrugghe B. Postnatal induction of transforming growth factor beta signaling in fibroblasts of mice recapitulates clinical, histologic, and biochemical features of scleroderma. Arthritis Rheum. 2007 Jan;56(1):334-44. PMID: 17195237

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 Feb 9;282(6):4007-20. Epub 2006 Dec 7 PMID: 17158461

Brandt-Bohne U, Keene DR, White FA, Koch M. MEGF9: a novel transmembrane protein with a strong and developmentally regulated expression in the nervous system. Biochem J. 2007 Jan 15;401(2):447-57. PMID: 16981854

Goldoni S, Iozzo RA, Kay P, Campbell S, McQuillan A, Agnew C, Zhu JX, Keene DR, Reed CC, Iozzo RV. A soluble ectodomain of LRIG1 inhibits cancer cell growth by attenuating basal and ligand-dependent EGFR activity. Oncogene. 2007 Jan 18;26(3):368-81. Epub 2006 Jul 17. PMID: 16847455

Boudko SP, Kuhn RJ, Rossmann MG. The coiled-coil domain structure of the Sin Nombre virus nucleocapsid protein. J Mol Biol. 2007 Mar 9;366(5):1538-44

Pryce BA, Brent AE, Murchison ND, Tabin CJ, Schweitzer R. Generation of transgenic tendon reporters, ScxGFP and ScxAP, using regulatory elements of the scleraxis gene. Dev Dyn. 2007 June:236(6):1677-82. PMID:17497702

Publications in 2006
Hooker, C. William and Hurlin, Peter J. Of Myc and Mnt. Journal of Cell Science, Solicited Review, 119: 208-216, 2006

Horton WA. Recent milestones in achondroplasia research. Am J Med Genet A. 2006 Jan 15;140A(2):166-169. PMID: 16353253

Dezfouli, S., Bakke, A., Huang, J., Wynshaw-Boris, A., Hurlin, PJ, Inflammatory disease and lymphomagenesis caused by deletion of the Myc antagonist Mnt in T cells. Mol Cell Biol. 2006 Mar;26(6):2080-92

 
Longobardi L, O'Rear L, Aakula S, Johnstone B, Shimer K, Chytil A, Horton WA, Moses HL, Spagnoli A. :Effect of IGF-I in the chondrogenesis of bone marrow mesenchymal stem cells in the presence or absence of TGF-beta signaling. J Bone Miner Res. 2006 Apr;21(4):626-36. Epub 2006 Apr 5 PMID: 16598383

Farnum CE, Lenox M, Zipfel W, Horton W, Williams R: In vivo delivery of fluoresceinated dextrans to the murine growth plate: imaging of three vascular routes by multiphoton micrsoscopy. Anat Rec A Discov Mol Cell Evol Biol 288:91-103, 2006

Dezfouli, F.S., Bakke, A, Wynshaw-Boris, A. and Hurlin, P.J. Disrupted T-cell homeostasis and lymphomagenesis caused by deletion of Mnt in T-cells. Mol. Cell. Biol. 26:2080-92. 2006

Whittaker MM, Mizuno K, Bachinger HP, Whittaker JW. Kinetic analysis of the metal binding mechanism of Escherichia coli manganese superoixde dismutase. Biophys J. 2006 Jan 15;90(2):598-607

Maria A. Schumacher, Kazunori Mizuno and Hans Peter Bächinger. "The crystal structure of a collagen-like polypeptide with 3(S)-hydroxyproline residues in the Xaa position forms a standard 7/2 collagen triple helix." J Biol Chem. 2006 Jun 23

Yoshihito Ishida, Hiroshi Kubota, Akitsugu Yamamoto, Akira Kitamura, Hans Peter Bächinger, Kazuhiro Nagata. "Type I Collagen in Hsp47-null cells is aggregated in ER and deficient in N-propeptide processing and fibrillogenesis." Mol Biol Cell. 2006 May;17(5):2346-55. Epub 2006 Mar 8

Peter J. Hurlin and Jie Huang. The MAX-interacting transcription factor network Seminars in Cancer Biology, Semin Cancer Biol. 2006 Jul 14

Toyo-oka K, Bowen TJ, Hirotsune S, Li Z, Jain S, Ota S, Escoubet-Lozach L, Garcia-Bassets I, Lozach J, Rosenfeld MG, Glass CK, Eisenman R, Ren B, Hurlin P, Wynshaw-Boris A. Mnt-deficient mammary glands exhibit impaired involution and tumors with characteristics of myc overexpression. Cancer Res. 2006 Jun 1;66(11):5565-73. Erratum in: Cancer Res. 2006 Jul 1;66(13):6894. Lozach, Laure Escoubet [corrected to Escoubet-Lozach, Laure]; Bassett, Ivan Garcia [corrected to Garcia-Bassets, Ivan]

Hoffman LM, Garcha K, Karamboulas K, Cowan MF, Drysdale LM, Horton WA, Underhill TM. BMP action in skeletogenesis involves attenuation of retinoid signaling. J Cell Biol. 2006 Jul 3;174(1):101-13

Veit G, Hansen U, Keene DR, Bruckner P, Chiquet-Ehrismann R, Chiquet M, Koch M. Collagen XII interacts with avian tenascin-X through its NC3 domain. J Biol Chem. 2006 Jul 21

Han R, Caswell CC, Lukomska E, Keene DR, Pawlowski M, Bujnicki JM, Kim JK, Lukomski S. Binding of the low-density lipoprotein by streptococcal collagen-like protein Scl1 of Streptococcus pyogenes. Mol Microbiol. 2006 Jul;61(2):351-67

Goldoni S, Iozzo RA, Kay P, Campbell S, McQuillan A, Agnew C, Zhu JX, Keene DR, Reed CC, Iozzo RV. A soluble ectodomain of LRIG1 inhibits cancer cell growth by attenuating basal and ligand-dependent EGFR activity. Oncogene. 2006 Jul 17 PMID 16847455

Carvalho HF, Felisbino SL, Keene DR, Vogel KG. Identification, content, and distribution of type VI collagen in bovine tendons. Cell Tissue Res. 2006 Aug;325(2):315-24. Epub 2006 Mar 28

Han R, Zwiefka A, Caswell CC, Xu Y, Keene DR, Lukomska E, Zhao Z, Hook M, Lukomski S. Assessment of prokaryotic collagen-like sequences derived from streptococcal Scl1 and Scl2 proteins as a source of recombinant GXY polymers. Appl Microbiol Biotechnol. 2006 Aug;72(1):109-15. Epub 2006 Mar 22

Woodley DT, Ram R, Doostan A, Bandyopadhyay P, Huang Y, Remington J, Hou Y, Keene DR, Liu Z, Chen M. Induction of epidermolysis bullosa acquisita in mice by passive transfer of autoantibodies from patients. J Invest Dermatol. 2006 Jun;126(6):1323-30

Carta L, Pereira 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. 2006 Mar

Veit G, Kobbe B, Keene DR, Paulsson M, Koch M, Wagener R. Collagen XXVIII, a novel von Willebrand factor A domain-containing protein with many imperfections in the collagenous domain. J. Biol. Chem. 2006 Feb 10;281(6):3494-504. Epub 2005 Dec 5 PMID: 16330543

DeLaurier, A., Schweitzer, R., Logan, M. Pitx1 determines the morphology of muscle, tendon, and bones of the hindlimb. Dev Biol. 2006 Nov 1;299(1):22-34. Epub 2006 Jul 14

Brandt-Bohne U, Keene DR, White FA, Koch, M, EGFL5: a novel transmembrane protein with a strong and developmentally regulated expression in the nervous system. Biochem J. 2006 Sep 18

Horton, WA, Molecular Pathogenesis of Achondroplasia., GGH. 2006 Dec; Vol. 22, No. 4

Horton, WA, Skeletal Development, Cell, Signaling and Growth, Wiley-VCH, Vol. 2: 619-40, 2006

Kuo CL, Isogai Z, Keene DR, Hazaki N, Ono RN, Sengle G, Bächinger, HP, Sakai LY, Effects of fibrillin-1 degradation on microfibril ultrastructure., J Biol Chem. 2006 Dec 7

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

Akiyama H, Stadler HS, Martin JF, Ishii TM, Beachy PA, Nakamura T, de Crombrugghe B., Misexpression of Sox9 in mouse limb bud mesenchyme induces polydactyly and rescues hypodactyly mice. Matrix Biol. 2006 Dec 8

Publications in 2005
Schumacher, M., Mizuno, K., and Bächinger, HP. The crystal structure of the collagen-like polypeptide (Glycyl-4(R)-hydroxyprolyl-4(R)-hydroxyprolyl)9 at 1.55 A resolution shows up-puckering of the praline ring in the Xaa position. J Biol Chem, 2005 Mar 22

Huan, J.; Meza-Romero, R.; Mooney, J.; Chou, Y.K.; Edwards, D.M.; Rich, C.; Link, J.M.; Vandenbark, A.A.; Bourdette, D.N.; Bächinger, H.P.; and Burrows, G.G. Rationally Designed Mutations Convert Complexes of Human Recombinant T Cell Receptor Ligands Into Monomers that Retain Biological Activity. Journal of Chemical Technology and Biotechnology. Vol 80 (1):pgs 2-12, 2005

Burrows, G.G.; Chang, J.W.; Huan, J.; Meza-Romero, R.; Mooney, J.; and Bächinger, H.P. Design, Engineering and Characterization of Human Recombinant T Cell Receptor Ligands Derived from Human Leukocyte Antigens DP2 and DP4. Journal of Chemical Technology and Biotechnology. Vol 247, April 2005

Jianya Y. Huan, Roberto Meza-Romero, Jeffery L. Mooney, Yuan K. Chou, David Edwards, Arthur A. Vandenbark, Hans Peter Bächinger, and Gregory G. Burrows. “Rationally Designed Mutations Convert Complexes of Human Recombinant T Cell Receptor Ligands Into Monomers that Retain Biological Activity. 2005

William Walker, Zi-Qiang Zhou, Sara Ota, Anthony Wynshaw-Boris and Peter J. Hurlin. Mnt:Max to Myc:Max complex switching regulates cell cycle entry. Journal of Cell Biology, 2005

Ortiz-Urba, 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. 2005 Mar 18; 307(5716): 1773-6

Humtsoe, JO., Kim, JK., Xu, Y., Keene, DR., Hook, M., Lukomski, S., Wary, KK. A streptococcal collagen-like protein interacts with the alpha 2beta 1 integrin and induces intracellular signaling. Journal of Biological Chemistry. 2005 Jan 12

Holden, P., Keene, DR., Lunstrum, GP., Bächinger, HP., Horton, WA. Secretion of cartilage oligomeric matrix protein is affected by the signal peptide. Journal of Biological Chemistry. 2005 Mar 3

Publications in 2004
Harms, M.J.; Wilmarth, P.A.; Kapfer, D.M.; Steel, E.A.; David, L.L.; Bächinger, H.P.; and Lampi, K.J. Laser light-scattering evidence for an altered association of {beta}B1-crystallin deamidated in the connecting peptide. Protein Sci, 13(3):678-686, 2004

Mizuno, K.; Hayashi, T.; Peyton, D.H.; and Bächinger, H.P. The peptides acetyl-(Gly-3(S)Hyp-4(R)Hyp)10-NH2 and acetyl-(Gly-Pro-3(S)Hyp)10-NH2 do not form a collagen triple helix. J Biol Chem, 279(1):282-7, 2004

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

Marneros, A.G.; Keene, D.R.; Hansen, U.; Fukai, N.; Moulton, K.; Goletz, P.L.; Moiseyev, G.; Pawlyk, B.S.; Halfter, W.; Dong, S.; Shibata, M.; Li, T.; Crouch, R.K.; Bruckner, P.; and Olsen, B.R. Collagen XVIII/endostatin is essential for vision and retinal pigment epithelial function. Embo J, 23(1):89-99, 2004

Hurlin, P.J.; Zhou, Z.Q.; Toyo-Oka, K.; Ota, S.; Walker, W.L.; Hirotsune, S.; and Wynshaw-Boris, A. Evidence of mnt-myc antagonism revealed by mnt gene deletion. Cell Cycle, 3(2):97-9, 2004

Hurlin, P. J.; Dezfouli, Shala. Functions of Myc:Max in the Control of Cell Proliferation and Tumorgenesis. International Review of Cytology, Vol. 238, 183-226, 2004

Cho, J.Y.; Guo, C.; Torello, M.; Lunstrum, G.P.; Iwata, T.; Deng, C.; and Horton, W.A. Defective lysosomal targeting of activated fibroblast growth factor receptor 3 in achondroplasia. Proc Natl Acad Sci U S A, 101(2):609-14, 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 Res Part C Embryo Today, 72(1):37-50, 2004

Judge, D.P.; Biery, N.J.; Keene, D.R.; Geubtner, J.; Myers, L.; Huso, D.L.; Sakai, L.Y.; and Dietz, H.C. Evidence for a critical contribution of haploinsufficiency in the complex pathogenesis of Marfan syndrome. J Clin Invest, 114(2):172-81, 2004

Meier, S.; Häussinger, D.; Pokidysheva, E.; Bächinger, H.P.; and Grzesiek, S. Determination of a high-precision NMR structure of the minicollagen cysteine rich domain from Hydra and characterization of its disulfide bond formation. FEBS Lett, 569(1-3):112-6, 2004

Mizuno, K.; Hayashi, T.; Peyton, D.H.; and Bächinger, H.P. Hydroxylation-induced stabilization of the collagen triple helix: Acetyl-(Glycyl-4(R)-hydroxyprolyl-4(R)-hydroxyprolyl)10-NH2 forms a highly stable triple helix. J. Biol. Chem. 279:38072-38078, 2004

Mizuno, K.; Whittaker, M.M.; Bächinger, H.P.; and Whittaker, J.W. Calorimetric studies on the tight binding metal interactions of Escherichia coli manganese superoxide dismutase. J Biol Chem, 279(26):27339-44, 2004

Pokidysheva, E.; Milbradt, A.G.; Meier, S.; Renner, C.; Haussinger, D.; Bächinger, H.P.; Moroder, L.; Grzesiek, S.; Holstein, T.W.; Ozbek, S.; and Engel, J. The structure of the Cys-rich terminal domain of Hydra minicollagen, which is involved in disulfide networks of the nematocyst wall. J Biol Chem, 279(29):30395-401, 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(22):23615-21, 2004

Koch M, Schulze J, Hansen U, Ashwodt T, Keene DR, Brunken WJ, Burgeson RE, Bruckner P, Bruckner-Tuderman L.: A novel marker of tissue junctions: Collagen XXII. J Biol Chem. 279:22514-22, 2004

Woodley DT, Keene DR, Atha T, Huang Y, Lipman K, Li W, Chen M:.Injection of recombinant human type VII collagen restores collagen function in dystrophic epidermolysis bullosa. Nat Med. 10(7):693-5, 2004

Woodley DT, Keene DR, Atha T, Huang Y, Ram R, Kasahara N, Chen M. Intradermal injection of lentiviral vectors corrects regenerated human dystrophic epidermolysis bullosa skin tissue in vivo. Mol Ther. 10:318-26, 2004

Bächinger, H.P., and Engel, J. “Thermodynamics and Kinetics of Collagen Folding.” 2004. Handbook of Protein Folding, Buchner, J. / Kiefhaber, T. (eds.) pp. 1055-1106. ISBN: 3-527-30784-2 (2004)

Knosp, W.M., Scott, V., Bächinger, H.P., and Stadler, H.S. HOXA13 directly regulates the expression of bone morphogenetic proteins 2 and 7 to control distal limb morphogenesis. Development, 131:4581-4592, 2004

Tavella S, Biticchi R, Schito A, Minina E, Di Martino D, Pagano A, Vortkamp A, Horton WA, Cancedda R, Garofalo S. Targeted expression of SHH affects chondrocyte differentiation, growth plate organization, and Sox9 expression. J Bone Miner Res 19(10):1678-88, 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, 2004

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