Mutations in B3GALT6, which Encodes a Glycosaminoglycan Linker Region Enzyme, Cause a Spectrum of Skeletal and Connective Tissue Disorders By exome sequencing, researchers found that B3GALT6 encoding an enzyme involved in the biosynthesis of the glycosaminoglycan linker region is responsible for a severe skeletal dysplasia, spondyloepimetaphyseal dysplasia with joint laxity type 1. [Am J Hum Genet] Abstract | Press Release Topical Application of Recombinant Type VII Collagen Incorporates Into the Dermal-Epidermal Junction and Promotes Wound Closure Patients with recessive dystrophic epidermolysis bullosa have incurable skin fragility, blistering, and skin wounds due to mutations in the gene that codes for type VII collagen (C7) that mediates dermal-epidermal adherence in human skin. Here the authors evaluated if topically applied human recombinant C7 could restore C7 at the dermal-epidermal junction and enhance wound healing. [Mol Ther] Abstract Semaphorin 3C Is a Novel Adipokine Linked to Extracellular Matrix Composition Alterations in white adipose tissue (WAT) function, including changes in protein (adipokine) secretion and extracellular matrix composition, promote an insulin-resistant state. Investigators set out to identify novel adipokines regulated by body fat mass in human subcutaneous WAT with potential roles in adipose function. [Diabetologia] Abstract Defective Endochondral Ossification-Derived Matrix and Bone Cells Alter the Lymphopoietic Niche in Collagen X Mouse Models Despite the appreciated interdependence of skeletal and hematopoietic development, the cell and matrix components of the hematopoietic niche remain to be fully defined. Utilizing mice with disrupted function of collagen X, a major hypertrophic cartilage matrix protein associated with endochondral ossification, the authors’ data identified a cytokine defect in trabecular bone cells at the chondro-osseous hematopoietic niche as a cause for aberrant B lymphopoiesis in these mice. [Stem Cells Dev] Abstract Adenosine A2A Receptors Promote Collagen Production by a Fli1- and CTGF-Mediated Mechanism Since expression of A2A receptors is increased in scleroderma fibroblasts, researchers examined the mechanisms by which the A2A receptor produces its fibrogenic effects. The effects of A2A receptor ligation on the expression of the transcription factor, Fli1, a constitutive repressor for the synthesis of matrix proteins, such as collagen, is studied in dermal fibroblasts. [Arthritis Res Ther] Abstract | Full Article Inhaled Steroids Modulate Extracellular Matrix Composition in Bronchial Biopsies of COPD Patients: A Randomized, Controlled Trial Investigators compared major extracellular matrix components (elastic fibers, proteoglycans, collagens type I and III) in bronchial biopsies after 30-months inhaled steroids treatment or placebo; and between current and ex-smokers with chronic obstructive pulmonary disease (COPD). [PLoS One] Full Article Overexpression of Smad7 Suppressed ROS/MMP9-Dependent Collagen Synthesis through Regulation of Heme Oxygenase-1 Scientists discovered that NADPH p47phox-dependent reactive oxygen species (ROS) production contributed to MMP9 activation and collagen expression, which was suppressed by transfecting pcDNA3-Smad7 in cardiac fibroblasts. [Mol Biol Rep] Abstract Extracellular Matrix Proteins Regulate Epithelial-Mesenchymal Transition in Mammary Epithelial Cells In rigid microenvironments, matrix metalloproteinase-3 (MMP3) upregulates expression of Rac1b, which translocates to the cell membrane to promote induction of reactive oxygen species and epithelial-mesenchymal transition (EMT). Investigators examined the role of the extracellular matrix (ECM) in this process. Their data showed that the basement membrane protein laminin suppresses the EMT response in MMP3-treated cells, whereas fibronectin promotes EMT. [Differentiation] Abstract In Vivo Biocompatibility and Biodegradation of a Novel Thin and Mechanically Stable Collagen Scaffold Scientists evaluated the in vivo biocompatibility and biodegradation of a novel, thin, mechanically stable and chemically non-crosslinked collagen cell carrier. [J Biomed Mater Res A] Abstract Tuning Scaffold Mechanics by Laminating Native Extracellular Matrix Membranes and Effects on Early Cellular Remodeling At approximately 50 μm thin, the human amniotic membrane (hAM) has been shown to be a versatile biomaterial with applications ranging from ocular transplants to skin and nerve regeneration. These investigations describe laminating layers of the hAM into a multilayered, conformation creating a thicker, more robust biomaterial for applications requiring more supportive structures. [J Biomed Mater Res A] Abstract |