FBN1: The disease-causing gene for Marfan syndrome and other genetic disorders von Sakai, Lynn Y., Keene, Douglas R., Renard, Marjolijn, De Backer, Julie

Volltext

Conversion of Mechanical Force into TGF-β-Mediated Biochemical Signals von Maeda, Toru, Sakabe, Tomoya, Sunaga, Ataru, Sakai, Keiko, Rivera, Alexander L., Keene, Douglas R., Sasaki, Takako, Stavnezer, Edward, Iannotti, Joseph, Schweitzer, Ronen, Ilic, Dusko, Baskaran, Harihara, Sakai, Takao

Volltext

Rapid fabrication of vascularized and innervated cell-laden bone models with biomimetic intrafibrillar collagen mineralization von Thrivikraman, Greeshma, Athirasala, Avathamsa, Gordon, Ryan, Zhang, Limin, Bergan, Raymond, Keene, Douglas R., Jones, James M., Xie, Hua, Chen, Zhiqiang, Tao, Jinhui, Wingender, Brian, Gower, Laurie, Ferracane, Jack L., Bertassoni, Luiz E.

Volltext

Novel Model of Tendon Regeneration Reveals Distinct Cell Mechanisms Underlying Regenerative and Fibrotic Tendon Healing von Howell, Kristen, Chien, Chun, Bell, Rebecca, Laudier, Damien, Tufa, Sara F., Keene, Douglas R., Andarawis-Puri, Nelly, Huang, Alice H.

Volltext

In vivo topical gene therapy for recessive dystrophic epidermolysis bullosa: a phase 1 and 2 trial von Gurevich, Irina, Agarwal, Pooja, Zhang, PeiPei, Dolorito, John A., Oliver, Stacie, Liu, Henry, Reitze, Nicholas, Sarma, Nikhil, Bagci, Isin Sinem, Sridhar, Kunju, Kakarla, Visesha, Yenamandra, Vamsi K., O’Malley, Mark, Prisco, Marco, Tufa, Sara F., Keene, Douglas R., South, Andrew P., Krishnan, Suma M., Marinkovich, M. Peter

Volltext

Creation and characterization of novel rat model for recessive dystrophic epidermolysis bullosa: Frameshift mutation of the Col7a1 gene leads to severe blistered phenotype von Stone, William, Strege, Chloe, Miller, William, Geurts, Aron M, Grzybowski, Michael, Riddle, Megan, Lees, Christopher, Eide, Cindy, Keene, Douglas R, Tufa, Sara F, Seelig, Davis, McGrath, John, Tolar, Jakub

Volltext

Fibrillin protein pleiotropy: Acromelic dysplasias von Sakai, Lynn Y., Keene, Douglas R.

Volltext

Type 2 alveolar cells are stem cells in adult lung von Barkauskas, Christina E, Cronce, Michael J, Rackley, Craig R, Bowie, Emily J, Keene, Douglas R, Stripp, Barry R, Randell, Scott H, Noble, Paul W, Hogan, Brigid L M

Volltext

Microenvironmental regulation by fibrillin-1 von Sengle, Gerhard, Tsutsui, Ko, Keene, Douglas R, Tufa, Sara F, Carlson, Eric J, Charbonneau, Noe L, Ono, Robert N, Sasaki, Takako, Wirtz, Mary K, Samples, John R, Fessler, Liselotte I, Fessler, John H, Sekiguchi, Kiyotoshi, Hayflick, Susan J, Sakai, Lynn Y

Volltext

Abnormal Activation of BMP Signaling Causes Myopathy in Fbn2 Null Mice von Sengle, Gerhard, Carlberg, Valerie, Tufa, Sara F, Charbonneau, Noe L, Smaldone, Silvia, Carlson, Eric J, Ramirez, Francesco, Keene, Douglas R, Sakai, Lynn Y

Volltext

The Fraser Complex Proteins (Frem1, Frem2, and Fras1) Can Form Anchoring Cords in the Absence of AMACO at the Dermal-Epidermal Junction of Mouse Skin von Esho, Temitope, Kobbe, Birgit, Tufa, Sara F, Keene, Douglas R, Paulsson, Mats, Wagener, Raimund

Volltext

Sex, pregnancy and aortic disease in Marfan syndrome von Renard, Marjolijn, Muiño-Mosquera, Laura, Manalo, Elise C, Tufa, Sara, Carlson, Eric J, Keene, Douglas R, De Backer, Julie, Sakai, Lynn Y

Volltext

Reversion‐inducing cysteine‐rich protein with Kazal motifs and MT1‐MMP promote the formation of robust fibrillin fibers von Matsuzaki, Tomoko, Keene, Douglas R., Nishimoto, Emi, Noda, Makoto

Volltext

Elevated TGFβ signaling contributes to ocular anterior segment dysgenesis in Col4a1 mutant mice von Mao, Mao, Labelle-Dumais, Cassandre, Tufa, Sara F., Keene, Douglas R., Gould, Douglas B.

Volltext

Lysyl hydroxylase 3–mediated post-translational modifications are required for proper biosynthesis of collagen α1α1α2(IV) von Ishikawa, Yoshihiro, Taga, Yuki, Coste, Thibault, Tufa, Sara F., Keene, Douglas R., Mizuno, Kazunori, Tournier-Lasserve, Elisabeth, Gould, Douglas B.

Volltext

QR-313, an Antisense Oligonucleotide, Shows Therapeutic Efficacy for Treatment of Dominant and Recessive Dystrophic Epidermolysis Bullosa: A Preclinical Study von Bornert, Olivier, Hogervorst, Marieke, Nauroy, Pauline, Bischof, Johannes, Swildens, Jim, Athanasiou, Ioannis, Tufa, Sara F., Keene, Douglas R., Kiritsi, Dimitra, Hainzl, Stefan, Murauer, Eva M., Marinkovich, M. Peter, Platenburg, Gerard, Hausser, Ingrid, Wally, Verena, Ritsema, Tita, Koller, Ulrich, Haisma, Elisabeth M., Nyström, Alexander

Volltext

Tgfβ signaling is critical for maintenance of the tendon cell fate von Tan, Guak-Kim, Pryce, Brian A, Stabio, Anna, Brigande, John V, Wang, ChaoJie, Xia, Zheng, Tufa, Sara F, Keene, Douglas R, Schweitzer, Ronen

Volltext

Regulation of tendon differentiation by scleraxis distinguishes force-transmitting tendons from muscle-anchoring tendons von Murchison, Nicholas D, Price, Brian A, Conner, David A, Keene, Douglas R, Olson, Eric N, Tabin, Clifford J, Schweitzer, Ronen

Volltext

Safety and Wound Outcomes Following Genetically Corrected Autologous Epidermal Grafts in Patients With Recessive Dystrophic Epidermolysis Bullosa von Siprashvili, Zurab, Nguyen, Ngon T, Gorell, Emily S, Loutit, Kylie, Khuu, Phuong, Furukawa, Louise K, Lorenz, H. Peter, Leung, Thomas H, Keene, Douglas R, Rieger, Kerri E, Khavari, Paul, Lane, Alfred T, Tang, Jean Y, Marinkovich, M. Peter

Volltext

Bone Marrow Transplantation for Recessive Dystrophic Epidermolysis Bullosa von Wagner, John E, Ishida-Yamamoto, Akemi, McGrath, John A, Hordinsky, Maria, Keene, Douglas R, Woodley, David T, Chen, Mei, Riddle, Megan J, Osborn, Mark J, Lund, Troy, Dolan, Michelle, Blazar, Bruce R, Tolar, Jakub

Volltext