Shriners Research Center, Portland OR

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Horton Laboratory | Bächinger Laboratory | Sakai Laboratory | Hurlin Laboratory | Stadler Laboratory | Schweitzer Laboratory | Keene Laboratory
6th Floor Laboratory Space | Electron Microscope | Confocal Imager
William Horton | Hans Peter Bächinger | Lynn Sakai | Peter Hurlin | Scott Stadler | Ronen Schweitzer | Douglas Keene
6th Pan Pacific Connective Tissue Symposium | Skeletal Growth Workshop

Keene Laboratory

CURRICULUM VITAE

Douglas R. Keene
Assistant Investigator, Imaging Center Director, Research Center
Shriners Hospitals for Children -- Portland
Instructor of Molecular & Medical Genetics
Oregon Health & Science University

INSTITUTION AND LOCATION DEGREE STUDY
Juniata College, Huntington, PA, Biology, B.S., 1977

HONORS
Past President, Northwest Microscopy Society; Faculty, UBC Cryo-TEM workshop.

RESEARCH AND/OR PROFESSIONAL EXPERIENCE
1985-Present   Shriners Hospitals for Children Research, Portland Unit, Portland, OR. Assistant Investigator, Director EM Facility.
1984-1985   Shriners Hospitals for Children Research, Portland Unit, Portland, OR. Research Associate.
1983-1984   Yale University, Departments of Biology/Molecular Biochemisty and Biophysics. Lecturer/Supervisor, EM Facility, New Haven, CT.
1981-1983   Yale University, Departments of Biology/Molecular Biochemistry and Biophysics. Acting Instructor/Supervisor, EM Facility, New Haven, CT.
1979-1981   Yale University, Departments of Biology/Molecular Biochemistry and Biophysics. Research Associate/Supervisor, EM Facility, New Haven, CT.
1978-1979   Yale University School of Medicine, Department of Physiology, New Haven, CT. Research Associate.
1977-1978   The Wistar Institute of Anatomy and Biology, Philadelphia, PA. Research Assistant.

PUBLICATIONS - Original Articles

1: Keene DR. A method for producing dust-, streak- and hole-free Formvar films in laboratories having high atmospheric humidity. Stain Technol. 1984 Jan;59(1):56-8. No abstract available. PMID: 6382684 [PubMed - indexed for MEDLINE]

2: Burgeson RE, Morris NP, Murray LW, Duncan KG, Keene DR, Sakai LY. The structure of type VII collagen. Ann N Y Acad Sci. 1985;460:47-57. No abstract available. PMID: 3868959 [PubMed - indexed for MEDLINE]

3: Morris NP, Keene DR, Glanville RW, Bentz H, Burgeson RE. The tissue form of type VII collagen is an antiparallel dimer. J Biol Chem. 1986 Apr 25;261(12):5638-44. PMID: 3082888 [PubMed - indexed for MEDLINE]

4: Lunstrum GP, Sakai LY, Keene DR, Morris NP, Burgeson RE. Large complex globular domains of type VII procollagen contribute to the structure of anchoring fibrils. J Biol Chem. 1986 Jul 5;261(19):9042-8. PMID: 3013874 [PubMed - indexed for MEDLINE]

5: Sakai LY, Keene DR, Morris NP, Burgeson RE. Type VII collagen is a major structural component of anchoring fibrils. J Cell Biol. 1986 Oct;103(4):1577-86. PMID: 3771648 [PubMed - indexed for MEDLINE]

6: Sakai LY, Keene DR, Engvall E. Fibrillin, a new 350-kD glycoprotein, is a component of extracellular microfibrils. J Cell Biol. 1986 Dec;103(6 Pt 1):2499-509. PMID: 3536967 [PubMed - indexed for MEDLINE]

7: Campagnone R, Regan J, Rich CB, Miller M, Keene DR, Sakai L, Foster JA. Pulmonary fibroblasts: a model system for studying elastin synthesis. Lab Invest. 1987 Feb;56(2):224-30. PMID: 3807319 [PubMed - indexed for MEDLINE]

8: Keene DR, Sakai LY, Burgeson RE, Bachinger HP. Direct visualization of IgM antibodies bound to tissue antigens using a monoclonal anti-type III collagen IgM as a model system. J Histochem Cytochem. 1987 Mar;35(3):311-8. PMID: 3546481 [PubMed - indexed for MEDLINE]

9: Keene DR, Sakai LY, Lunstrum GP, Morris NP, Burgeson RE. Type VII collagen forms an extended network of anchoring fibrils. J Cell Biol. 1987 Mar;104(3):611-21. PMID: 3818794 [PubMed - indexed for MEDLINE]

10: Lunstrum GP, Kuo HJ, Rosenbaum LM, Keene DR, Glanville RW, Sakai LY, Burgeson RE. Anchoring fibrils contain the carboxyl-terminal globular domain of type VII procollagen, but lack the amino-terminal globular domain. J Biol Chem. 1987 Oct 5;262(28):13706-12. PMID: 2443495 [PubMed - indexed for MEDLINE]

11: Keene DR, Sakai LY, Bachinger HP, Burgeson RE. Type III collagen can be present on banded collagen fibrils regardless of fibril diameter. J Cell Biol. 1987 Nov;105(5):2393-402. PMID: 2445760 [PubMed - indexed for MEDLINE]

12: Keene DR. Enhanced visualization of delicate macromolecules by negative staining followed by low angle rotary shadowing. J Electron Microsc Tech. 1987 Nov;7(3):233-4. No abstract available. PMID: 2462029 [PubMed - indexed for MEDLINE]

13: Foster JA, Rich CB, Horrigan S, Miller M, Dadd CA, Baule V, Keene DR. Synthesis of soluble and insoluble elastins in cultures of chick aortic cells. Lab Invest. 1988 Jun;58(6):667-73. PMID: 3379914 [PubMed - indexed for MEDLINE]

14: Keene DR, Engvall E, Glanville RW. Ultrastructure of type VI collagen in human skin and cartilage suggests an anchoring function for this filamentous network. J Cell Biol. 1988 Nov;107(5):1995-2006. PMID: 3182942 [PubMed - indexed for MEDLINE]

15: Godfrey M, Keene DR, Blank E, Hori H, Sakai LY, Sherwin LA, Hollister DW. Type II achondrogenesis-hypochondrogenesis: morphologic and immunohistopathologic studies. Am J Hum Genet. 1988 Dec;43(6):894-903. PMID: 3057886 [PubMed - indexed for MEDLINE]

16: Kuo HJ, Keene DR, Glanville RW. Orientation of type VI collagen monomers in molecular aggregates. Biochemistry. 1989 May 2;28(9):3757-62. PMID: 2502170 [PubMed - indexed for MEDLINE]

17: Maddox BK, Sakai LY, Keene DR, Glanville RW. Connective tissue microfibrils. Isolation and characterization of three large pepsin-resistant domains of fibrillin. J Biol Chem. 1989 Dec 15;264(35):21381-5. PMID: 2512293 [PubMed - indexed for MEDLINE]

18: Burgeson RE, Lunstrum GP, Rokosova B, Rimberg CS, Rosenbaum LM, Keene DR. The structure and function of type VII collagen. Ann N Y Acad Sci. 1990;580:32-43. Review. No abstract available. PMID: 2186694 [PubMed - indexed for MEDLINE]

19: Wirtz MK, Keene DR, Hori H, Glanville RW, Steinmann B, Rao VH, Hollister DW. In vivo and in vitro noncovalent association of excised alpha 1 (I) amino-terminal propeptides with mutant pN alpha 2(I) collagen chains in native mutant collagen in a case of Ehlers-Danlos syndrome, type VII. J Biol Chem. 1990 Apr 15;265(11):6312-7. PMID: 2318855 [PubMed - indexed for MEDLINE]

20: Bachinger HP, Morris NP, Lunstrum GP, Keene DR, Rosenbaum LM, Compton LA, Burgeson RE. The relationship of the biophysical and biochemical characteristics of type VII collagen to the function of anchoring fibrils. J Biol Chem. 1990 Jun 15;265(17):10095-101. PMID: 2112541 [PubMed - indexed for MEDLINE]

21: Keene DR, Sakai LY, Burgeson RE. Human bone contains type III collagen, type VI collagen, and fibrillin: type III collagen is present on specific fibers that may mediate attachment of tendons, ligaments, and periosteum to calcified bone cortex. J Histochem Cytochem. 1991 Jan;39(1):59-69. PMID: 1983874 [PubMed - indexed for MEDLINE]

22: Keene DR, Maddox BK, Kuo HJ, Sakai LY, Glanville RW. Extraction of extendable beaded structures and their identification as fibrillin-containing extracellular matrix microfibrils. J Histochem Cytochem. 1991 Apr;39(4):441-9. PMID: 2005373 [PubMed - indexed for MEDLINE]

23: Lunstrum GP, Morris NP, McDonough AM, Keene DR, Burgeson RE. Identification and partial characterization of two type XII-like collagen molecules. J Cell Biol. 1991 May;113(4):963-9. PMID: 2026655 [PubMed - indexed for MEDLINE]

24: Keene DR, Lunstrum GP, Morris NP, Stoddard DW, Burgeson RE. Two type XII-like collagens localize to the surface of banded collagen fibrils. J Cell Biol. 1991 May;113(4):971-8. PMID: 2026656 [PubMed - indexed for MEDLINE]

25: Rousselle P, Lunstrum GP, Keene DR, Burgeson RE. Kalinin: an epithelium-specific basement membrane adhesion molecule that is a component of anchoring filaments. J Cell Biol. 1991 Aug;114(3):567-76. PMID: 1860885 [PubMed - indexed for MEDLINE]

26: Sakai LY, Keene DR, Glanville RW, Bachinger HP. Purification and partial characterization of fibrillin, a cysteine-rich structural component of connective tissue microfibrils. J Biol Chem. 1991 Aug 5;266(22):14763-70. PMID: 1860873 [PubMed - indexed for MEDLINE]

27: Wang H, Paul R, Burgeson RE, Keene DR, Kabat D. Plasma membrane receptors for ecotropic murine retroviruses require a limiting accessory factor. J Virol. 1991 Dec;65(12):6468-77. PMID: 1942243 [PubMed - indexed for MEDLINE]

28: Hori H, Keene DR, Sakai LY, Wirtz MK, Bachinger HP, Godfrey M, Hollister DW. Repeated helical epitopes of defined amino acid sequence in human type III collagen identified by monoclonal antibodies. Mol Immunol. 1992 Jun;29(6):759-70. PMID: 1376414 [PubMed - indexed for MEDLINE]

29: Lunstrum GP, McDonough AM, Marinkovich MP, Keene DR, Morris NP, Burgeson RE. Identification and partial purification of a large, variant form of type XII collagen. J Biol Chem. 1992 Oct 5;267(28):20087-92. PMID: 1400326 [PubMed - indexed for MEDLINE]

30: Watt SL, Lunstrum GP, McDonough AM, Keene DR, Burgeson RE, Morris NP. Characterization of collagen types XII and XIV from fetal bovine cartilage. J Biol Chem. 1992 Oct 5;267(28):20093-9. PMID: 1400327 [PubMed - indexed for MEDLINE]

31: Marinkovich MP, Lunstrum GP, Keene DR, Burgeson RE. The dermal-epidermal junction of human skin contains a novel laminin variant. J Cell Biol. 1992 Nov;119(3):695-703. PMID: 1383241 [PubMed - indexed for MEDLINE]

32: Marinkovich MP, Keene DR, Rimberg CS, Burgeson RE. Cellular origin of the dermal-epidermal basement membrane. Dev Dyn. 1993 Aug;197(4):255-67. PMID: 8292823 [PubMed - indexed for MEDLINE]

33: Keene DR, McDonald K. The ultrastructure of the connective tissue matrix of skin and cartilage after high-pressure freezing and freeze-substitution. J Histochem Cytochem. 1993 Aug;41(8):1141-53. PMID: 8331280 [PubMed - indexed for MEDLINE]

34: Marinkovich MP, Verrando P, Keene DR, Meneguzzi G, Lunstrum GP, Ortonne JP, Burgeson RE. Basement membrane proteins kalinin and nicein are structurally and immunologically identical. Lab Invest. 1993 Sep;69(3):295-9. PMID: 8377472 [PubMed - indexed for MEDLINE]

35: Sakai LY, Keene DR. Fibrillin: monomers and microfibrils. Methods Enzymol. 1994;245:29-52. No abstract available. PMID: 7760739 [PubMed - indexed for MEDLINE]

36: Nelson RE, Fessler LI, Takagi Y, Blumberg B, Keene DR, Olson PF, Parker CG, Fessler JH. Peroxidasin: a novel enzyme-matrix protein of Drosophila development. EMBO J. 1994 Aug 1;13(15):3438-47. PMID: 8062820 [PubMed - indexed for MEDLINE]

37: Rosati R, Horan GS, Pinero GJ, Garofalo S, Keene DR, Horton WA, Vuorio E, de Crombrugghe B, Behringer RR. Normal long bone growth and development in type X collagen-null mice. Nat Genet. 1994 Oct;8(2):129-35. PMID: 7842010 [PubMed - indexed for MEDLINE]

38: Andrikopoulos K, Liu X, Keene DR, Jaenisch R, Ramirez F. Targeted mutation in the col5a2 gene reveals a regulatory role for type V collagen during matrix assembly. Nat Genet. 1995 Jan;9(1):31-6. PMID: 7704020 [PubMed - indexed for MEDLINE]

39: Roark EF, Keene DR, Haudenschild CC, Godyna S, Little CD, Argraves WS. The association of human fibulin-1 with elastic fibers: an immunohistological, ultrastructural, and RNA study. J Histochem Cytochem. 1995 Apr;43(4):401-11. PMID: 7534784 [PubMed - indexed for MEDLINE]

40: Kuo HJ, Keene DR, Glanville RW. The macromolecular structure of type-VI collagen. Formation and stability of filaments. Eur J Biochem. 1995 Sep 1;232(2):364-72. PMID: 7556183 [PubMed - indexed for MEDLINE]

41: Keene DR, Oxford JT, Morris NP. Ultrastructural localization of collagen types II, IX, and XI in the growth plate of human rib and fetal bovine epiphyseal cartilage: type XI collagen is restricted to thin fibrils. J Histochem Cytochem. 1995 Oct;43(10):967-79. PMID: 7560887 [PubMed - indexed for MEDLINE]

42: Champliaud MF, Lunstrum GP, Rousselle P, Nishiyama T, Keene DR, Burgeson RE. Human amnion contains a novel laminin variant, laminin 7, which like laminin 6, covalently associates with laminin 5 to promote stable epithelial-stromal attachment. J Cell Biol. 1996 Mar;132(6):1189-98. PMID: 8601594 [PubMed - indexed for MEDLINE]

43: Marinkovich MP, Taylor TB, Keene DR, Burgeson RE, Zone JJ. LAD-1, the linear IgA bullous dermatosis autoantigen, is a novel 120-kDa anchoring filament protein synthesized by epidermal cells. J Invest Dermatol. 1996 Apr;106(4):734-8. Erratum in: J Invest Dermatol 1996 Jun;106(6):1343. PMID: 8618013 [PubMed - indexed for MEDLINE]

44: Reinhardt DP, Keene DR, Corson GM, Poschl E, Bachinger HP, Gambee JE, Sakai LY. Fibrillin-1: organization in microfibrils and structural properties. J Mol Biol. 1996 Apr 26;258(1):104-16. PMID: 8613981 [PubMed - indexed for MEDLINE]

45: Mechling DE, Gambee JE, Morris NP, Sakai LY, Keene DR, Mayne R, Bachinger HP. Type IX collagen NC1 domain peptides can trimerize in vitro without forming a triple helix. J Biol Chem. 1996 Jun 7;271(23):13781-5. PMID: 8662808 [PubMed - indexed for MEDLINE]

46: Trotter JA, Lyons-Levy G, Luna D, Koob TJ, Keene DR, Atkinson MA. Stiparin: a glycoprotein from sea cucumber dermis that aggregates collagen fibrils. Matrix Biol. 1996 Jul;15(2):99-110. PMID: 8837011 [PubMed - indexed for MEDLINE]

47: Takagi Y, Nomizu M, Gullberg D, MacKrell AJ, Keene DR, Yamada Y, Fessler JH. Conserved neuron promoting activity in Drosophila and vertebrate laminin alpha1. J Biol Chem. 1996 Jul 26;271(30):18074-81. PMID: 8663504 [PubMed - indexed for MEDLINE]

48: Reinhardt DP, Sasaki T, Dzamba BJ, Keene DR, Chu ML, Gohring W, Timpl R, Sakai LY. Fibrillin-1 and fibulin-2 interact and are colocalized in some tissues. J Biol Chem. 1996 Aug 9;271(32):19489-96. PMID: 8702639 [PubMed - indexed for MEDLINE]

49: Maddox BK, Garofalo S, Smith C, Keene DR, Horton WA. Skeletal development in transgenic mice expressing a mutation at Gly574Ser of type II collagen. Dev Dyn. 1997 Feb;208(2):170-7. PMID: 9022054 [PubMed - indexed for MEDLINE]

50: Reinhardt DP, Mechling DE, Boswell BA, Keene DR, Sakai LY, Bachinger HP. Calcium determines the shape of fibrillin. J Biol Chem. 1997 Mar 14;272(11):7368-73. PMID: 9054436 [PubMed - indexed for MEDLINE]

51: Keene DR, Jordan CD, Reinhardt DP, Ridgway CC, Ono RN, Corson GM, Fairhurst M, Sussman MD, Memoli VA, Sakai LY. Fibrillin-1 in human cartilage: developmental expression and formation of special banded fibers. J Histochem Cytochem. 1997 Aug;45(8):1069-82. PMID: 9267468 [PubMed - indexed for MEDLINE]

52: Maddox BK, Garofalo S, Keene DR, Smith C, Horton WA. Type II collagen pro-alpha-chains containing a Gly574Ser mutation are not incorporated into the cartilage matrix of transgenic mice. Matrix Biol. 1997 Aug;16(3):93-103. PMID: 9314159 [PubMed - indexed for MEDLINE]

53: Rousselle P, Keene DR, Ruggiero F, Champliaud MF, Rest M, Burgeson RE. Laminin 5 binds the NC-1 domain of type VII collagen. J Cell Biol. 1997 Aug 11;138(3):719-28. PMID: 9245798 [PubMed - indexed for MEDLINE]

54: Keene DR, Marinkovich MP, Sakai LY. Immunodissection of the connective tissue matrix in human skin. Microsc Res Tech. 1997 Aug 15;38(4):394-406. Review. PMID: 9297689 [PubMed - indexed for MEDLINE]

55: Kuo HJ, Maslen CL, Keene DR, Glanville RW. Type VI collagen anchors endothelial basement membranes by interacting with type IV collagen. J Biol Chem. 1997 Oct 17;272(42):26522-9. PMID: 9334230 [PubMed - indexed for MEDLINE]

56: Pereira L, Andrikopoulos K, Tian J, Lee SY, Keene DR, Ono R, Reinhardt DP, Sakai LY, Biery NJ, Bunton T, Dietz HC, Ramirez F. Targetting of the gene encoding fibrillin-1 recapitulates the vascular aspect of Marfan syndrome. Nat Genet. 1997 Oct;17(2):218-22. PMID: 9326947 [PubMed - indexed for MEDLINE]

57: Maddox BK, Keene DR, Sakai LY, Charbonneau NL, Morris NP, Ridgway CC, Boswell BA, Sussman MD, Horton WA, Bachinger HP, Hecht JT. The fate of cartilage oligomeric matrix protein is determined by the cell type in the case of a novel mutation in pseudoachondroplasia. J Biol Chem. 1997 Dec 5;272(49):30993-7. PMID: 9388247 [PubMed - indexed for MEDLINE]

58: Keene DR, Ridgway CC, Iozzo RV. Type VI microfilaments interact with a specific region of banded collagen fibrils in skin. J Histochem Cytochem. 1998 Feb;46(2):215-20. PMID: 9446828 [PubMed - indexed for MEDLINE]

59: Lunstrum GP, Keene DR, Weksler NB, Cho YJ, Cornwall M, Horton WA. Chondrocyte differentiation in a rat mesenchymal cell line. J Histochem Cytochem. 1999 Jan;47(1):1-6. PMID: 9857207 [PubMed - indexed for MEDLINE]

60: Forlino A, Keene DR, Schmidt K, Marini JC. An alpha2(I) glycine to aspartate substitution is responsible for the presence of a kink in type I collagen in a lethal case of osteogenesis imperfecta. Matrix Biol. 1998 Dec;17(8-9):575-84. PMID: 9923651 [PubMed - indexed for MEDLINE]

61: Zhu Y, Oganesian A, Keene DR, Sandell LJ. Type IIA procollagen containing the cysteine-rich amino propeptide is deposited in the extracellular matrix of prechondrogenic tissue and binds to TGF-beta1 and BMP-2. J Cell Biol. 1999 Mar 8;144(5):1069-80. PMID: 10085302 [PubMed - indexed for MEDLINE]

62: Trotter JA, Lyons-Levy G, Chino K, Koob TJ, Keene DR, Atkinson MA. Collagen fibril aggregation-inhibitor from sea cucumber dermis. Matrix Biol. 1999 Dec;18(6):569-78. PMID: 10607918 [PubMed - indexed for MEDLINE]

63: Dallas SL, Keene DR, Bruder SP, Saharinen J, Sakai LY, Mundy GR, Bonewald LF. Role of the latent transforming growth factor beta binding protein 1 in fibrillin-containing microfibrils in bone cells in vitro and in vivo. J Bone Miner Res. 2000 Jan;15(1):68-81. PMID: 10646116 [PubMed - indexed for MEDLINE]

64: Amano S, Scott IC, Takahara K, Koch M, Champliaud MF, Gerecke DR, Keene DR, Hudson DL, Nishiyama T, Lee S, Greenspan DS, Burgeson RE. Bone morphogenetic protein 1 is an extracellular processing enzyme of the laminin 5 gamma 2 chain. J Biol Chem. 2000 Jul 28;275(30):22728-35. PMID: 10806203 [PubMed - indexed for MEDLINE]

65: Morris NP, Oxford JT, Davies GB, Smoody BF, Keene DR. Developmentally regulated alternative splicing of the alpha1(XI) collagen chain: spatial and temporal segregation of isoforms in the cartilage of fetal rat long bones. J Histochem Cytochem. 2000 Jun;48(6):725-41. PMID: 10820146 [PubMed - indexed for MEDLINE]

66: Keene DR, San Antonio JD, Mayne R, McQuillan DJ, Sarris G, Santoro SA, Iozzo RV. Decorin binds near the C terminus of type I collagen. J Biol Chem. 2000 Jul 21;275(29):21801-4. PMID: 10823816 [PubMed - indexed for MEDLINE]

67: Gayraud B, Keene DR, Sakai LY, Ramirez F. New insights into the assembly of extracellular microfibrils from the analysis of the fibrillin 1 mutation in the tight skin mouse. J Cell Biol. 2000 Aug 7;150(3):667-80. PMID: 10931876 [PubMed - indexed for MEDLINE]

68: Raff ML, Craigen WJ, Smith LT, Keene DR, Byers PH. Partial COL1A2 gene duplication produces features of osteogenesis imperfecta and Ehlers-Danlos syndrome type VII. Hum Genet. 2000 Jan;106(1):19-28. PMID: 10982177 [PubMed - indexed for MEDLINE]

69: Xu Y, Gurusiddappa S, Rich RL, Owens RT, Keene DR, Mayne R, Hook A, Hook M. Multiple binding sites in collagen type I for the integrins alpha1beta1 and alpha2beta1. J Biol Chem. 2000 Dec 15;275(50):38981-9. PMID: 10986291 [PubMed - indexed for MEDLINE]

70: Fitzgerald J, Morgelin M, Selan C, Wiberg C, Keene DR, Lamande SR, Bateman JF. The N-terminal N5 subdomain of the alpha 3(VI) chain is important for collagen VI microfibril formation. J Biol Chem. 2001 Jan 5;276(1):187-93. PMID: 11027693 [PubMed - indexed for MEDLINE]

71: Koch M, Murrell JR, Hunter DD, Olson PF, Jin W, Keene DR, Brunken WJ, Burgeson RE. A novel member of the netrin family, beta-netrin, shares homology with the beta chain of laminin: identification, expression, and functional characterization. J Cell Biol. 2000 Oct 16;151(2):221-34. PMID: 11038171 [PubMed - indexed for MEDLINE]

72: Maddox BK, Mokashi A, Keene DR, Bachinger HP. A cartilage oligomeric matrix protein mutation associated with pseudoachondroplasia changes the structural and functional properties of the type 3 domain. J Biol Chem. 2000 Apr 14;275(15):11412-7. PMID: 10753957 [PubMed - indexed for MEDLINE]

73: Chen M, Keene DR, Costa FK, Tahk SH, Woodley DT. The carboxyl terminus of type VII collagen mediates antiparallel dimer formation and constitutes a new antigenic epitope for epidermolysis Bullosa acquisita autoantibodies. J Biol Chem. 2001 Jun 15;276(24):21649-55. Epub 2001 Mar 27. PMID: 11274208 [PubMed - indexed for MEDLINE]

74: Vranka J, Mokashi A, Keene DR, Tufa S, Corson G, Sussman M, Horton WA, Maddox K, Sakai L, Bachinger HP. Selective intracellular retention of extracellular matrix proteins and chaperones associated with pseudoachondroplasia. Matrix Biol. 2001 Nov;20(7):439-50. PMID: 11691584 [PubMed - indexed for MEDLINE]

75: Gregory KE, Keene DR, Tufa SF, Lunstrum GP, Morris NP. Developmental distribution of collagen type XII in cartilage: association with articular cartilage and the growth plate. J Bone Miner Res. 2001 Nov;16(11):2005-16. PMID: 11697796 [PubMed - indexed for MEDLINE]

76: Isogai Z, Aspberg A, Keene DR, Ono RN, Reinhardt DP, Sakai LY. Versican interacts with fibrillin-1 and links extracellular microfibrils to other connective tissue networks. J Biol Chem. 2002 Feb 8;277(6):4565-72. Epub 2001 Nov 28. PMID: 11726670 [PubMed - indexed for MEDLINE]

77: Gaiser KG, Maddox BK, Bann JG, Boswell BA, Keene DR, Garofalo S, Horton WA. Y-position collagen II mutation disrupts cartilage formation and skeletal development in a transgenic mouse model of spondyloepiphyseal dysplasia. J Bone Miner Res. 2002 Jan;17(1):39-47. PMID: 11771668 [PubMed - indexed for MEDLINE]

78: Moller T, Franch T, Hojrup P, Keene DR, Bachinger HP, Brennan RG, Valentin-Hansen P. Hfq: a bacterial Sm-like protein that mediates RNA-RNA interaction. Mol Cell. 2002 Jan;9(1):23-30. PMID: 11804583 [PubMed - indexed for MEDLINE]

79: Hirohata S, Wang LW, Miyagi M, Yan L, Seldin MF, Keene DR, Crabb JW, Apte SS. Punctin, a novel ADAMTS-like molecule, ADAMTSL-1, in extracellular matrix. J Biol Chem. 2002 Apr 5;277(14):12182-9. Epub 2002 Jan 22. PMID: 11805097 [PubMed - indexed for MEDLINE]

80: Dzamba BJ, Keene DR, Isogai Z, Charbonneeau NL, Karaman-Jurukovska N, Simon M, Sakai LY. Assembly of epithelial cell fibrillins. J Invest Dermatol. 2001 Dec;117(6):1612-20. PMID: 11886530

81: Fukai N, Eklund L, Marneros AG, Oh SP, Keene DR, Tamarkin L, Niemela M, Ilves M, Li E, Pihlajaniemi T, Olsen BR. Lack of collagen XVIII/endostatin results in eye abnormalities. EMBO J. 2002 Apr 2;21(7):1535-44. PMID: 11927538 [PubMed - indexed for MEDLINE]

82: Xu Y, Keene DR, Bujnicki JM, Hook M, Lukomski S. Streptococcal Scl1 and Scl2 proteins form collagen-like triple helices. J Biol Chem. 2002 Jul 26;277(30):27312-8. Epub 2002 Apr 25. PMID: 11976327 [PubMed - indexed for MEDLINE]

83: Rattenholl A, Pappano WN, Koch M, Keene DR, Kadler KE, Sasaki T, Timpl R, Burgeson RE, Greenspan DS, Bruckner-Tuderman L. Proteinases of the bone morphogenetic protein-1 family convert procollagen VII to mature anchoring fibril collagen. J Biol Chem. 2002 Jul 19;277(29):26372-8. Epub 2002 May 1. PMID: 11986329 [PubMed - indexed for MEDLINE]

84: Ortiz-Urda S, Thyagarajan B, Keene DR, Lin Q, Fang M, Calos MP, Khavari PA. Stable nonviral genetic correction of inherited human skin disease. Nat Med. 2002 Oct;8(10):1166-70. Epub 2002 Sep 16. Erratum in: Nat Med. 2003 Feb;9(2):237. PMID: 12244305 [PubMed - indexed for MEDLINE]

85: Steiglitz BM, Keene DR, Greenspan DS. PCOLCE2 encodes a functional procollagen C-proteinase enhancer (PCPE2) that is a collagen-binding protein differing in distribution of expression and post-translational modification from the previously described PCPE1. J Biol Chem. 2002 Dec 20;277(51):49820-30. Epub 2002 Oct 20. PMID: 12393877 [PubMed - indexed for MEDLINE]

86: Chen M, Kasahara N, Keene DR, Chan L, Hoeffler WK, Finlay D, Barcova M, Cannon PM, Mazurek C, Woodley DT. Restoration of type VII collagen expression and function in dystrophic epidermolysis bullosa. Nat Genet. 2002 Dec;32(4):670-5. Epub 2002 Nov 11. PMID: 12426566 [PubMed - indexed for MEDLINE]

87: Isogai Z, Ono RN, Ushiro S, Keene DR, Chen Y, Mazzieri R, Charbonneau NL, Reinhardt DP, Rifkin DB, Sakai LY. Latent transforming growth factor beta-binding protein 1 interacts with fibrillin and is a microfibril-associated protein. J Biol Chem. 2003 Jan 24;278(4):2750-7. Epub 2002 Nov 11. PMID: 12429738 [PubMed - indexed for MEDLINE]

88: Charbonneau NL, Dzamba BJ, Ono RN, Keene DR, Corson GM, Reinhardt DP, Sakai LY. Fibrillins can co-assemble in fibrils, but fibrillin fibril composition displays cell-specific differences. J Biol Chem. 2003 Jan 24;278(4):2740-9. Epub 2002 Nov 11. PMID: 12429739 [PubMed - indexed for MEDLINE]

89: Veitch DP, Nokelainen P, McGowan KA, Nguyen TT, Nguyen NE, Stephenson R, Pappano WN, Keene DR, Spong SM, Greenspan DS, Findell PR, Marinkovich MP. Mammalian tolloid metalloproteinase, and not matrix metalloprotease 2 or membrane type 1 metalloprotease, processes laminin-5 in keratinocytes and skin. J Biol Chem. 2003 May 2;278(18):15661-8. Epub 2002 Dec 7. PMID: 12473650 [PubMed - indexed for MEDLINE]

90: Ortiz-Urda S, Lin Q, Green CL, Keene DR, Marinkovich MP, Khavari PA. Injection of genetically engineered fibroblasts corrects regenerated human epidermolysis bullosa skin tissue. J Clin Invest. 2003 Jan;111(2):251-5. PMID: 12531881 [PubMed - indexed for MEDLINE]

91: Li J, Tzu J, Chen Y, Zhang YP, Nguyen NT, Gao J, Bradley M, Keene DR, Oro AE, Miner JH, Marinkovich MP. Laminin-10 is crucial for hair morphogenesis. EMBO J. 2003 May 15;22(10):2400-10. PMID: 12743034 [PubMed - indexed for MEDLINE]

92: Pappano WN, Steiglitz BM, Scott IC, Keene DR, Greenspan DS. Use of Bmp1/Tll1 doubly homozygous null mice and proteomics to identify and validate in vivo substrates of bone morphogenetic protein 1/tolloid-like metalloproteinases. Mol Cell Biol. 2003 Jul;23(13):4428-38. PMID: 12808086 [PubMed - indexed for MEDLINE]

93: Ortiz-Urda S, Thyagarajan B, Keene DR, Lin Q, Calos MP, Khavari PA. PhiC31 integrase-mediated nonviral genetic correction of junctional epidermolysis bullosa. Hum Gene Ther. 2003 Jun 10;14(9):923-8. PMID: 12828862 [PubMed - indexed for MEDLINE]

94: Marneros AG, Keene DR, Hansen U, Fukai N, Moulton K, Goletz PL, Moiseyev G, Pawlyk BS, Halfter W, Dong S, Shibata M, Li T, Crouch RK, Bruckner P, Olsen BR. Collagen XVIII/endostatin is essential for vision and retinal pigment epithelial function. EMBO J. 2004 Jan 14;23(1):89-99. Epub 2003 Dec 11. PMID: 14685281 [PubMed - indexed for MEDLINE]

95: Woodley DT, Krueger GG, Jorgensen CM, Fairley JA, Atha T, Huang Y, Chan L, Keene DR, Chen M. Normal and gene-corrected dystrophic epidermolysis bullosa fibroblasts alone can produce type VII collagen at the basement membrane zone. J Invest Dermatol. 2003 Nov;121(5):1021-8. PMID: 14708601 [PubMed - indexed for MEDLINE]

96: Corson GM, Charbonneau NL, Keene DR, Sakai LY. Differential expression of fibrillin-3 adds to microfibril variety in human and avian, but not rodent, connective tissues. Genomics. 2004 Mar;83(3):461-72. PMID: 14962672 [PubMed - indexed for MEDLINE]

97: 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. 2004 May 21;279(21):22514-21. Epub 2004 Mar 10. PMID: 15016833 [PubMed - indexed for MEDLINE]

98: Charbonneau NL, Ono RN, Corson GM, Keene DR, Sakai LY. Fine tuning of growth factor signals depends on fibrillin microfibril networks. Birth Defects Res C Embryo Today. 2004 Mar;72(1):37-50. Review. PMID: 15054903 [PubMed - indexed for MEDLINE]

99: 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. 2004 Jul;10(7):693-5. Epub 2004 Jun 13. PMID: 15195089 [PubMed - indexed for MEDLINE]

100: Judge DP, Biery NJ, Keene DR, Geubtner J, Myers L, Huso DL, Sakai LY, Dietz HC. Evidence for a critical contribution of haploinsufficiency in the complex pathogenesis of Marfan syndrome. J Clin Invest. 2004 Jul;114(2):172-81. PMID: 15254584 [PubMed - indexed for MEDLINE]

101: 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. 2004 Aug;10(2):318-26. PMID: 15294178 [PubMed - indexed for MEDLINE]

102: Humtsoe JO, Kim JK, Xu Y, Keene DR, Hook M, Lukomski S, Wary KK. A streptococcal collagen-like protein interacts with the alpha2beta1 integrin and induces intracellular signaling. J Biol Chem. 2005 Apr 8;280(14):13848-57. Epub 2005 Jan 12. PMID: 15647274 [PubMed - indexed for MEDLINE]

103: Holden P, Keene DR, Lunstrum GP, Bachinger HP, Horton WA. Secretion of cartilage oligomeric matrix protein is affected by the signal peptide. J Biol Chem. 2005 Apr 29;280(17):17172-9. Epub 2005 Mar 3. PMID: 15749701 [PubMed - indexed for MEDLINE]

104: Poon CJ, Plaas AH, Keene DR, McQuillan DJ, Last K, Fosang AJ. N-linked keratan sulfate in the aggrecan interglobular domain potentiates aggrecanase activity. J Biol Chem. 2005 Jun 24;280(25):23615-21. Epub 2005 Apr 22. PMID: 15849197 [PubMed - indexed for MEDLINE]

105: Battista S, Guarnieri D, Borselli C, Zeppetelli S, Borzacchiello A, Mayol L, Gerbasio D, Keene DR, Ambrosio L, Netti PA. The effect of matrix composition of 3D constructs on embryonic stem cell differentiation. Biomaterials. 2005 Nov;26(31):6194-207. PMID: 15921736 [PubMed - indexed for MEDLINE]

106: Gregory KE, Ono RN, Charbonneau NL, Kuo CL, Keene DR, Bachinger HP, Sakai LY. The prodomain of BMP-7 targets the BMP-7 complex to the extracellular matrix. J Biol Chem. 2005 Jul 29;280(30):27970-80. Epub 2005 Jun 1. PMID: 15929982 [PubMed - indexed for MEDLINE]

107: Kim JK, Xu Y, Xu X, Keene DR, Gurusiddappa S, Liang X, Wary KK, Hook M. A novel binding site in collagen type III for integrins alpha1beta1 and alpha2beta1. J Biol Chem. 2005 Sep 16;280(37):32512-20. Epub 2005 Jul 21. PMID: 16043429 [PubMed - indexed for MEDLINE]

108: 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 [PubMed - indexed for MEDLINE]

109: Zong Y, Xu Y, Liang X, Keene DR, Hook A, Gurusiddappa S, Hook M, Narayana SV. A 'Collagen Hug' model for Staphylococcus aureus CNA binding to collagen. EMBO J. 2005 Dec 21;24(24):4224-36. Epub 2005 Dec 15. Erratum in: EMBO J. 2006 Feb 22;25(4):921. PMID: 16362049 [PubMed - indexed for MEDLINE]

110: 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 24;281(12):8016-23. Epub 2005 Dec 28. PMID: 16407178 [PubMed - indexed for MEDLINE]

111: 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. PMID: 16543893 [PubMed - indexed for MEDLINE]

112: 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. PMID: 16552563 [PubMed - indexed for MEDLINE]

113: 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. PMID: 16568302 [PubMed - indexed for MEDLINE]

114: 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 [PubMed - indexed for MEDLINE]

115: 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. PMID: 16856940 [PubMed - indexed for MEDLINE]

116: 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 Sep 15;281(37):27461-70. Epub 2006 Jul 21. PMID: 16861231 [PubMed - indexed for MEDLINE]

117: 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 [PubMed - indexed for MEDLINE]

118: Kuo CL, Isogai Z, Keene DR, Hazeki N, Ono RN, Sengle G, Peter Bachinger H, 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 [PubMed - indexed for MEDLINE]

119: 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 [PubMed - indexed for MEDLINE]

120: 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. PMID: 17206620 [PubMed - indexed for MEDLINE]

121: 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 [PubMed - indexed for MEDLINE]

122: 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. 2007 Apr;282(14):10647-59. Epub 2007 Feb 8. PMID: 17293346 [PubMed - indexed for MEDLINE]

123: 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. 2007 May;26(4):314-23. Epub 2007 Jan 18. PMID: 17307347 [PubMed - indexed for MEDLINE]

124: Guarnieri D, Battista S, Borzacchiello A, Mayol L, De Rosa E, Keene DR, Muscariello L, Barbarisi A, Netti PA. 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 [PubMed - indexed for MEDLINE]

125: 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 [PubMed - indexed for MEDLINE]

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

127: 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 [PubMed - as supplied by publisher]

128: Murchison ND, Price 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 [PubMed - indexed for MEDLINE]

129:  Caswell CC, Han R, Hovis KM, Ciborowski P, Keene DR, Marconi RT, Lukomski S.  The Sc11 protein of M6-type group A Streptococcus binds the human complement regulatory protein, factor H, and inhibits the alternative pathway of complement.  Mol Microbiol. 2008 Feb; 67(3):584-96. Epub 2007 Dec 18.  PMID:  18093091

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

131: 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]

132:  Russell BH, Vasan R, Keene DR, Koehler TM, Xu Y., Potential dissemination of Bacillus anthracis utilizing human lung epithelial cells. Cell Microbiol. 2008 Apr;10(4):945-57. Epub 2007 Dec 6. PMID: 18067609 [PubMed - in process]

133: Shaut, C.A.E., Keene, D.R., Sorensen, L., Li, D.Y. and Stadler H.S, A novel role for  HOXA13 in placental development and labyrinth endothelial specification, PLoS Genetics, PLoS Genet. 2008 May 16;4(5):e1000073.  PMID: 18483557

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