Douglas R. Keene

Lack of collagen XVIII/endostatin results in eye abnormalities

Fukai, Naomi, Eklund, Lauri, Marneros, Alexander G., Oh, Suk Paul, Keene, Douglas R., Tamarkin, Lawrence, ...

Mice lacking collagen XVIII and its proteolytically derived product endostatin show delayed regression of blood vessels in the vitreous along the surface of the retina after birth and lack of or...

Injection of genetically engineered fibroblasts corrects regenerated human epidermolysis bullosa skin tissue

Ortiz-Urda, Susana, Lin, Qun, Green, Cheryl L., Keene, Douglas R., Marinkovich, M. Peter, Khavari, Paul A.

Current therapeutic strategies for genetic skin disorders rely on the complex process of grafting genetically engineered tissue to recipient wound beds. Because fibroblasts synthesize and secrete...

Laminin-10 is crucial for hair morphogenesis

Li, Jie, Tzu, Julia, Chen, Yi, Zhang, Yan-Ping, Nguyen, Ngon T., Gao, Jing, ...

The role of the extracellular matrix in cutaneous morphogenesis is poorly understood. Here, we describe the essential role of laminin-10 (α5β1γ1) in hair follicle development. Laminin-10 was...

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

Pappano, William N., Steiglitz, Barry M., Scott, Ian C., Keene, Douglas R., Greenspan, Daniel S.

Bone morphogenetic protein 1 (BMP-1) and mammalian Tolloid (mTLD), two proteinases encoded by Bmp1, provide procollagen C-proteinase (pCP) activity that converts procollagens I to III into the major...

Evidence for a critical contribution of haploinsufficiency in the complex pathogenesis of Marfan syndrome

Judge, Daniel P., Biery, Nancy J., Keene, Douglas R., Geubtner, Jessica, Myers, Loretha, Huso, David L., ...

Marfan syndrome is a connective tissue disorder caused by mutations in the gene encoding fibrillin-1 (FBN1). A dominant-negative mechanism has been inferred based upon dominant inheritance,...

Collagen XVIII/endostatin is essential for vision and retinal pigment epithelial function

Marneros, Alexander G, Keene, Douglas R, Hansen, Uwe, Fukai, Naomi, Moulton, Karen, Goletz, Patrice L, ...

Age-related macular degeneration (ARMD) with abnormal deposit formation under the retinal pigment epithelium (RPE) is the major cause of blindness in the Western world. basal laminar deposits are...

A ‘Collagen Hug' Model for Staphylococcus aureus CNA binding to collagen

Zong, Yinong, Xu, Yi, Liang, Xiaowen, Keene, Douglas R, Höök, Agneta, Gurusiddappa, Shivasankarappa, ...

The structural basis for the association of eukaryotic and prokaryotic protein receptors and their triple-helical collagen ligand remains poorly understood. Here, we present the crystal structures of...

Lack of collagen XVIII/endostatin results in eye abnormalities

Fukai, Naomi, Eklund, Lauri, Marneros, Alexander G., Oh, Suk Paul, Keene, Douglas R., Tamarkin, Lawrence, ...

Mice lacking collagen XVIII and its proteolytically derived product endostatin show delayed regression of blood vessels in the vitreous along the surface of the retina after birth and lack of or...

Injection of genetically engineered fibroblasts corrects regenerated human epidermolysis bullosa skin tissue

Ortiz-Urda, Susana, Lin, Qun, Green, Cheryl L., Keene, Douglas R., Marinkovich, M. Peter, Khavari, Paul A.

Current therapeutic strategies for genetic skin disorders rely on the complex process of grafting genetically engineered tissue to recipient wound beds. Because fibroblasts synthesize and secrete...

Laminin-10 is crucial for hair morphogenesis

Li, Jie, Tzu, Julia, Chen, Yi, Zhang, Yan-Ping, Nguyen, Ngon T., Gao, Jing, ...

The role of the extracellular matrix in cutaneous morphogenesis is poorly understood. Here, we describe the essential role of laminin-10 (α5β1γ1) in hair follicle development. Laminin-10 was...

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

Pappano, William N., Steiglitz, Barry M., Scott, Ian C., Keene, Douglas R., Greenspan, Daniel S.

Bone morphogenetic protein 1 (BMP-1) and mammalian Tolloid (mTLD), two proteinases encoded by Bmp1, provide procollagen C-proteinase (pCP) activity that converts procollagens I to III into the major...

Evidence for a critical contribution of haploinsufficiency in the complex pathogenesis of Marfan syndrome

Judge, Daniel P., Biery, Nancy J., Keene, Douglas R., Geubtner, Jessica, Myers, Loretha, Huso, David L., ...

Marfan syndrome is a connective tissue disorder caused by mutations in the gene encoding fibrillin-1 (FBN1). A dominant-negative mechanism has been inferred based upon dominant inheritance,...

Collagen XVIII/endostatin is essential for vision and retinal pigment epithelial function

Marneros, Alexander G, Keene, Douglas R, Hansen, Uwe, Fukai, Naomi, Moulton, Karen, Goletz, Patrice L, ...

Age-related macular degeneration (ARMD) with abnormal deposit formation under the retinal pigment epithelium (RPE) is the major cause of blindness in the Western world. basal laminar deposits are...

A ‘Collagen Hug' model for Staphylococcus aureus CNA binding to collagen

Zong, Yinong, Xu, Yi, Liang, Xiaowen, Keene, Douglas R, Höök, Agneta, Gurusiddappa, Shivasankarappa, ...

Correction to: The EMBO Journal (2005) 24, 4224–4236. doi:10.1038/sj.emboj.7600888

Laminin 5 Binds the NC-1 Domain of Type VII Collagen

Rousselle, Patricia, Keene, Douglas R., Ruggiero, Florence, Champliaud, Marie-France, Rest, Michel Van Der, Burgeson, Robert E.

Mutational analyses of genes that encode components of the anchoring complex underlying the basolateral surface of external epithelia indicate that this structure is the major element providing for...

Type IIA Procollagen Containing the Cysteine-rich Amino Propeptide Is Deposited in the Extracellular Matrix of Prechondrogenic Tissue and Binds to TGF-β1 and BMP-2

Zhu, Yong, Oganesian, Anush, Keene, Douglas R., Sandell, Linda J.

Type II procollagen is expressed as two splice forms. One form, type IIB, is synthesized by chondrocytes and is the major extracellular matrix component of cartilage. The other form, type IIA,...

New Insights into the Assembly of Extracellular Microfibrils from the Analysis of the Fibrillin 1 Mutation in the Tight skin Mouse

Gayraud, Barbara, Keene, Douglas R., Sakai, Lynn Y., Ramirez, Francesco

The Tight skin (Tsk) mutation is a duplication of the mouse fibrillin 1 (Fbn1) gene that results in a larger (418 kD) than normal (350 kD) protein; Tsk/+ mice display increased connective tissue,...

A Novel Member of the Netrin Family, β-Netrin, Shares Homology with the β Chain of Laminin: Identification, Expression, and Functional Characterization

Koch, Manuel, Murrell, Julie R., Hunter, Dale D., Olson, Pamela F., Jin, William, Keene, Douglas R., ...

The netrins are a family of laminin-related molecules. Here, we characterize a new member of the family, β-netrin. β-Netrin is homologous to the NH2 terminus of laminin chain short arms; it...

HOXA13 Is Essential for Placental Vascular Patterning and Labyrinth Endothelial Specification

Shaut, Carley A. E., Keene, Douglas R., Sorensen, Lise K., Li, Dean Y., Stadler, H. Scott

In eutherian mammals, embryonic growth and survival is dependent on the formation of the placenta, an organ that facilitates the efficient exchange of oxygen, nutrients, and metabolic waste between...

Targeting of Bone Morphogenetic Protein Growth Factor Complexes to Fibrillin*

Sengle, Gerhard, Charbonneau, Noe L., Ono, Robert N., Sasaki, Takako, Alvarez, Jennifer, Keene, Douglas R., ...

Both latent transforming growth factor-β (TGF-β)-binding proteins fibrillins are components of microfibril networks, and both interact with members of the TGF-β family of growth factors....