Douglas R. Keene
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View article: Targeted incorporation of collagen IV to the basement membrane: A step forward for developing extracellular protein therapies
Targeted incorporation of collagen IV to the basement membrane: A step forward for developing extracellular protein therapies Open
The collagen IV scaffold serves as a fundamental structural unit of the basement membrane (BM). Understanding its structure, assembly, and function is essential for tissue engineering, the design of organoid models, and developing therapie…
View article: Biochemical characterization of collagen I in Warmblood Fragile Foal Syndrome horse lysyl hydroxylase 1 mutation.
Biochemical characterization of collagen I in Warmblood Fragile Foal Syndrome horse lysyl hydroxylase 1 mutation. Open
Mutations in the collagen-modifying enzyme lysyl hydroxylase 1 (LH1) cause Warmblood Fragile Foal Syndrome (WFFS) in horses. We investigated the impact of this mutation on collagen structure and function. Our results show that LH1 deficien…
View article: Creation and characterization of novel rat model for recessive dystrophic epidermolysis bullosa: Frameshift mutation of the Col7a1 gene leads to severe blistered phenotype
Creation and characterization of novel rat model for recessive dystrophic epidermolysis bullosa: Frameshift mutation of the Col7a1 gene leads to severe blistered phenotype Open
Recessive dystrophic epidermolysis bullosa is a rare genodermatosis caused by a mutation of the Col7a1 gene. The Col7a1 gene codes for collagen type VII protein, a major component of anchoring fibrils. Mutations of the Col7a1 gene can caus…
View article: Characterization of TGFβ1-induced tendon-like structure in the scaffold-free three-dimensional tendon cell culture system
Characterization of TGFβ1-induced tendon-like structure in the scaffold-free three-dimensional tendon cell culture system Open
The biological mechanisms regulating tenocyte differentiation and morphological maturation have not been well-established, partly due to the lack of reliable in vitro systems that produce highly aligned collagenous tissues. In this study, …
View article: Unraveling the role of TGFβ signaling in thoracic aortic aneurysm and dissection using Fbn1 mutant mouse models
Unraveling the role of TGFβ signaling in thoracic aortic aneurysm and dissection using Fbn1 mutant mouse models Open
View article: 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
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 Open
AMACO (VWA2 protein), secreted by epithelial cells, is strongly expressed at basement membranes when budding or invagination occurs in embryos. In skin, AMACO associates with proteins of the Fraser complex, which form anchoring cords. Thes…
View article: Lysyl hydroxylase 3–mediated post-translational modifications are required for proper biosynthesis of collagen α1α1α2(IV)
Lysyl hydroxylase 3–mediated post-translational modifications are required for proper biosynthesis of collagen α1α1α2(IV) Open
View article: EMILIN1 deficiency causes arterial tortuosity with osteopenia and connects impaired elastogenesis with defective collagen fibrillogenesis
EMILIN1 deficiency causes arterial tortuosity with osteopenia and connects impaired elastogenesis with defective collagen fibrillogenesis Open
EMILIN1 (elastin-microfibril-interface-located-protein-1) is a structural component of the elastic fiber network and localizes to the interface between the fibrillin microfibril scaffold and the elastin core. How EMILIN1 contributes to con…
View article: Vertebrate extracellular matrix protein hemicentin-1 interacts physically and genetically with basement membrane protein nidogen-2
Vertebrate extracellular matrix protein hemicentin-1 interacts physically and genetically with basement membrane protein nidogen-2 Open
View article: Characterization of TGFβ-induced tendon-like structure in scaffold-free three-dimensional tendon cell culture
Characterization of TGFβ-induced tendon-like structure in scaffold-free three-dimensional tendon cell culture Open
Tendons transmit mechanical forces between muscle and bone. Their biomechanical function requires high tensile strength provided by highly organized collagen fibers. Tenocytes mainly drive tendon growth via extracellular matrix (ECM) produ…
View article: Elevated TGFβ signaling contributes to ocular anterior segment dysgenesis in Col4a1 mutant mice
Elevated TGFβ signaling contributes to ocular anterior segment dysgenesis in Col4a1 mutant mice Open
Ocular anterior segment dysgenesis (ASD) refers to a collection of developmental disorders affecting the anterior structures of the eye. Although a number of genes have been implicated in the etiology of ASD, the underlying pathogenetic me…
View article: In vivo topical gene therapy for recessive dystrophic epidermolysis bullosa: a phase 1 and 2 trial
In vivo topical gene therapy for recessive dystrophic epidermolysis bullosa: a phase 1 and 2 trial Open
View article: A new murine model of Barth syndrome neutropenia links TAFAZZIN deficiency to increased ER stress-induced apoptosis
A new murine model of Barth syndrome neutropenia links TAFAZZIN deficiency to increased ER stress-induced apoptosis Open
Barth syndrome is an inherited X-linked disorder that leads to cardiomyopathy, skeletal myopathy, and neutropenia. These symptoms result from the loss of function of the enzyme TAFAZZIN, a transacylase located in the inner mitochondrial me…
View article: Cell autonomous TGFβ signaling is essential for stem/progenitor cell recruitment into degenerative tendons
Cell autonomous TGFβ signaling is essential for stem/progenitor cell recruitment into degenerative tendons Open
Understanding cell recruitment in damaged tendons is critical for improvements in regenerative therapy. We recently reported that targeted disruption of transforming growth factor beta (TGFβ) type II receptor in the tendon cell lineage (Tg…
View article: Molecular alterations due to <i>Col5a1</i> haploinsufficiency in a mouse model of classic Ehlers–Danlos syndrome
Molecular alterations due to <i>Col5a1</i> haploinsufficiency in a mouse model of classic Ehlers–Danlos syndrome Open
Type V collagen is a regulatory fibrillar collagen essential for type I collagen fibril nucleation and organization and its deficiency leads to structurally abnormal extracellular matrix (ECM). Haploinsufficiency of the Col5a1 gene encodin…
View article: The Good, the Bad and the Ugly: Task-Specific Fixation for Connective Tissues
The Good, the Bad and the Ugly: Task-Specific Fixation for Connective Tissues Open
An abstract is not available for this content so a preview has been provided. As you have access to this content, a full PDF is available via the ‘Save PDF’ action button.
View article: Induction of Epidermolysis Bullosa Acquisita in Mice by Passive Transfer of Autoantibodies from Patients
Induction of Epidermolysis Bullosa Acquisita in Mice by Passive Transfer of Autoantibodies from Patients Open
Epidermolysis bullosa acquisita (EBA) is an autoimmune sub-epidermal blistering disease characterized by autoantibodies to type VII (anchoring fibril) collagen. To date, however, direct evidence for a pathogenic role of human EBA autoantib…
View article: 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
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 Open
Our results suggest that missense variants in ECM genes may not cause catastrophic changes to the TM, but over many years, subtle changes in ECM may accumulate and cause structural disorganization of the outflow resistance leading to eleva…
View article: Localized chondro-ossification underlies joint dysfunction and motor deficits in the <i>Fkbp10</i> mouse model of osteogenesis imperfecta
Localized chondro-ossification underlies joint dysfunction and motor deficits in the <i>Fkbp10</i> mouse model of osteogenesis imperfecta Open
Significance Osteogenesis imperfecta (OI), also known as brittle bone disease, commonly features low bone mass and frequent fractures. We and others have previously identified mutations that cause joint deformities in OI patients. However,…
View article: Tendon and motor phenotypes in the Crtap-/- mouse model of recessive osteogenesis imperfecta
Tendon and motor phenotypes in the Crtap-/- mouse model of recessive osteogenesis imperfecta Open
Osteogenesis imperfecta (OI) is characterized by short stature, skeletal deformities, low bone mass, and motor deficits. A subset of OI patients also present with joint hypermobility; however, the role of tendon dysfunction in OI pathogene…
View article: Reticulocalbin 3 is involved in postnatal tendon development by regulating collagen fibrillogenesis and cellular maturation
Reticulocalbin 3 is involved in postnatal tendon development by regulating collagen fibrillogenesis and cellular maturation Open
View article: 438 A phase 1/2 trial of PTR-01, a collagen 7 (C7) protein replacement therapy, in patients with recessive dystrophic epidermolysis bullosa (RDEB)
438 A phase 1/2 trial of PTR-01, a collagen 7 (C7) protein replacement therapy, in patients with recessive dystrophic epidermolysis bullosa (RDEB) Open
View article: <i>Ezh2</i> Is Essential for Patterning of Multiple Musculoskeletal Tissues but Dispensable for Tendon Differentiation
<i>Ezh2</i> Is Essential for Patterning of Multiple Musculoskeletal Tissues but Dispensable for Tendon Differentiation Open
An efficient musculoskeletal system depends on the precise assembly and coordinated growth and function of muscles, skeleton, and tendons. However, the mechanisms that drive integrated musculoskeletal development and coordinated growth and…
View article: Author response: Tendon and motor phenotypes in the Crtap-/- mouse model of recessive osteogenesis imperfecta
Author response: Tendon and motor phenotypes in the Crtap-/- mouse model of recessive osteogenesis imperfecta Open
Article Figures and data Abstract Introduction Results Discussion Materials and methods Data availability References Decision letter Author response Article and author information Metrics Abstract Osteogenesis imperfecta (OI) is characteri…
View article: Type I and type V procollagen triple helix uses different subsets of the molecular ensemble for lysine posttranslational modifications in the rER
Type I and type V procollagen triple helix uses different subsets of the molecular ensemble for lysine posttranslational modifications in the rER Open
View article: Loss of Smad4 in the scleraxis cell lineage results in postnatal joint contracture
Loss of Smad4 in the scleraxis cell lineage results in postnatal joint contracture Open
View article: Cell autonomous TGF-beta signaling is essential for cell recruitment into degenerating tendons
Cell autonomous TGF-beta signaling is essential for cell recruitment into degenerating tendons Open
Understanding the role of cell recruitment in tendon disorders is critical for improvements in regenerative therapy. We recently reported that targeted disruption of TGFβ type II receptor in the tendon cell lineage ( Tgfbr2 ScxCre ) result…
View article: Type 2 alveolar cells are stem cells in adult lung
Type 2 alveolar cells are stem cells in adult lung Open
Gas exchange in the lung occurs within alveoli, air-filled sacs composed of type 2 and type 1 epithelial cells (AEC2s and AEC1s), capillaries, and various resident mesenchymal cells. Here, we use a combination of in vivo clonal lineage ana…
View article: Enhancer of Zeste Homolog 2 (<i>Ezh2</i>) is essential for patterning of multiple musculoskeletal tissues but dispensable for tendon differentiation
Enhancer of Zeste Homolog 2 (<i>Ezh2</i>) is essential for patterning of multiple musculoskeletal tissues but dispensable for tendon differentiation Open
An efficient musculoskeletal system depends on the precise assembly and coordinated growth and function of muscles, skeleton and tendons. However, the mechanisms that drive integrated musculoskeletal development and coordinated growth and …
View article: Immune tolerance of allogeneic haematopoietic cell transplantation supports donor epidermal grafting of recessive dystrophic epidermolysis bullosa chronic wounds*
Immune tolerance of allogeneic haematopoietic cell transplantation supports donor epidermal grafting of recessive dystrophic epidermolysis bullosa chronic wounds* Open
The immune tolerance established by alloHCT supports successful adoptive transfer of donor epidermal grafts. Persistence of donor grafts in a single patient beyond 1 year and observed migration of donor-grafted cells into adjacent wound su…