Search Results - "Tufa, Sara F"

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

“…To date, the cell and molecular mechanisms regulating tendon healing are poorly understood. Here, we establish a novel model of tendon regeneration using…”
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In vivo topical gene therapy for recessive dystrophic epidermolysis bullosa: a phase 1 and 2 trial by 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

“…Recessive dystrophic epidermolysis bullosa (RDEB) is a lifelong genodermatosis associated with blistering, wounding, and scarring caused by mutations in COL7A1…”
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QR-313, an Antisense Oligonucleotide, Shows Therapeutic Efficacy for Treatment of Dominant and Recessive Dystrophic Epidermolysis Bullosa: A Preclinical Study by 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

“…Dystrophic epidermolysis bullosa (DEB) is a blistering skin disease caused by mutations in the gene COL7A1 encoding collagen VII. DEB can be inherited as…”
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Tgfβ signaling is critical for maintenance of the tendon cell fate by Tan, Guak-Kim, Pryce, Brian A, Stabio, Anna, Brigande, John V, Wang, ChaoJie, Xia, Zheng, Tufa, Sara F, Keene, Douglas R, Schweitzer, Ronen

“…Studies of cell fate focus on specification, but little is known about maintenance of the differentiated state. In this study, we find that the mouse tendon…”
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Creation and characterization of novel rat model for recessive dystrophic epidermolysis bullosa: Frameshift mutation of the Col7a1 gene leads to severe blistered phenotype by 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

“…Recessive dystrophic epidermolysis bullosa is a rare genodermatosis caused by a mutation of the Col7a1 gene. The Col7a1 gene codes for collagen type VII…”
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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 by Esho, Temitope, Kobbe, Birgit, Tufa, Sara F, Keene, Douglas R, Paulsson, Mats, Wagener, Raimund

“…AMACO (VWA2 protein), secreted by epithelial cells, is strongly expressed at basement membranes when budding or invagination occurs in embryos. In skin, AMACO…”
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Abnormal Activation of BMP Signaling Causes Myopathy in Fbn2 Null Mice by Sengle, Gerhard, Carlberg, Valerie, Tufa, Sara F, Charbonneau, Noe L, Smaldone, Silvia, Carlson, Eric J, Ramirez, Francesco, Keene, Douglas R, Sakai, Lynn Y

“…Fibrillins are large extracellular macromolecules that polymerize to form the backbone structure of connective tissue microfibrils. Mutations in the gene for…”
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Loss of Smad4 in the scleraxis cell lineage results in postnatal joint contracture by Schlesinger, Saundra Y., Seo, Seongkyung, Pryce, Brian A., Tufa, Sara F., Keene, Douglas R., Huang, Alice H., Schweitzer, Ronen

“…Growth of the musculoskeletal system requires precise coordination between bone, muscle, and tendon during development. Insufficient elongation of the…”
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Musculoskeletal integration at the wrist underlies the modular development of limb tendons by Huang, Alice H, Riordan, Timothy J, Pryce, Brian, Weibel, Jennifer L, Watson, Spencer S, Long, Fanxin, Lefebvre, Veronique, Harfe, Brian D, Stadler, H Scott, Akiyama, Haruhiko, Tufa, Sara F, Keene, Douglas R, Schweitzer, Ronen

“…The long tendons of the limb extend from muscles that reside in the zeugopod (arm/leg) to their skeletal insertions in the autopod (paw). How these connections…”
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Microenvironmental regulation by fibrillin-1 by 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

“…Fibrillin-1 is a ubiquitous extracellular matrix molecule that sequesters latent growth factor complexes. A role for fibrillin-1 in specifying tissue…”
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Tendon and motor phenotypes in the Crtap -/- mouse model of recessive osteogenesis imperfecta by Grol, Matthew William, Haelterman, Nele A, Lim, Joohyun, Munivez, Elda M, Archer, Marilyn, Hudson, David M, Tufa, Sara F, Keene, Douglas R, Lei, Kevin, Park, Dongsu, Kuzawa, Cole D, Ambrose, Catherine G, Eyre, David R, Lee, Brendan H

“…Osteogenesis imperfecta (OI) is characterized by short stature, skeletal deformities, low bone mass, and motor deficits. A subset of OI patients also present…”
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Anchoring Cords: A Distinct Suprastructure in the Developing Skin by Esho, Temitope, Tufa, Sara F., Kobbe, Birgit, Wohl, Alexander P., Sengle, Gerhard, Paulsson, Mats, Keene, Douglas R., Wagener, Raimund

“…AMACO (VWA2 protein) is a basement membrane-associated protein secreted by epithelial cells. It is strongly expressed when invagination or budding occurs…”
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Elevated TGFβ signaling contributes to ocular anterior segment dysgenesis in Col4a1 mutant mice by Mao, Mao, Labelle-Dumais, Cassandre, Tufa, Sara F., Keene, Douglas R., Gould, Douglas B.

“…•Col4a1 mutant mice have anterior segment dysgenesis and other ocular defects affecting lens and retina.•TGFβ signaling is elevated in developing anterior…”
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Confocal/TEM overlay microscopy: a simple method for correlating confocal and electron microscopy of cells expressing GFP/YFP fusion proteins by Keene, Douglas R, Tufa, Sara F, Lunstrum, Gregory P, Holden, Paul, Horton, William A

“…Genetic manipulation allows simultaneous expression of green fluorescent protein (GFP) and its derivatives with a wide variety of cellular proteins in a…”
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Lysyl hydroxylase 3–mediated post-translational modifications are required for proper biosynthesis of collagen α1α1α2(IV) by Ishikawa, Yoshihiro, Taga, Yuki, Coste, Thibault, Tufa, Sara F., Keene, Douglas R., Mizuno, Kazunori, Tournier-Lasserve, Elisabeth, Gould, Douglas B.

“…Collagens are the most abundant proteins in the body and among the most biosynthetically complex. A molecular ensemble of over 20 endoplasmic reticulum…”
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Type I and type V procollagen triple helix uses different subsets of the molecular ensemble for lysine posttranslational modifications in the rER by Ishikawa, Yoshihiro, Taga, Yuki, Zientek, Keith, Mizuno, Nobuyo, Salo, Antti M., Semenova, Olesya, Tufa, Sara F., Keene, Douglas R., Holden, Paul, Mizuno, Kazunori, Gould, Douglas B., Myllyharju, Johanna, Bächinger, Hans Peter

“…Collagen is the most abundant protein in humans. It has a characteristic triple-helix structure and is heavily posttranslationally modified. The complex…”
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Connective Tissue Ultrastructure: A Direct Comparison between Conventional Specimen Preparation and High‐Pressure Freezing/Freeze‐Substitution by Keene, Douglas R., Tufa, Sara F.

“…ABSTRACT It is generally agreed within the microscopy community that the quality of ultrastructure within the connective tissue matrix resulting from…”
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Unraveling the role of TGFβ signaling in thoracic aortic aneurysm and dissection using Fbn1 mutant mouse models by Deleeuw, Violette, Carlson, Eric, Renard, Marjolijn, Zientek, Keith D., Wilmarth, Phillip A., Reddy, Ashok P., Manalo, Elise C., Tufa, Sara F., Keene, Douglas R., Olbinado, Margie, Stampanoni, Marco, Kanki, Sachiko, Yanagisawa, Hiromi, Mosquera, Laura Muiño, Sips, Patrick, De Backer, Julie, Sakai, Lynn Y.

“…•Reducing the expression of Tgfb2 or replacing wildtype Fbn1 with a mutant allele in which the first hybrid domain is deleted showed comparable deleterious…”
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Identification of Missense Extracellular Matrix Gene Variants in a Large Glaucoma Pedigree and Investigation of the N700S Thrombospondin-1 Variant in Normal and Glaucomatous Trabecular Meshwork Cells by Wirtz, Mary K., Sykes, Renee, Samples, John, Edmunds, Beth, Choi, Dongseok, Keene, Douglas R., Tufa, Sara F., Sun, Ying Ying, Keller, Kate E.

“…Primary open-angle glaucoma (POAG) is a complex heterogeneous disease. While several POAG genes have been identified, a high proportion of estimated…”
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