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TGF-β is the prototypical profibrotic cytokine, which can, by itself, stimulate the synthesis of ECM components by myofibroblasts and up-regulate the transcription of TGF-β target genes via nuclear accumulation of phosphorylated SMAD2/3 (pSMAD2/3) in complex with SMAD4 ( 6). These cells mostly originate from resident, quiescent, or resting fibroblasts in response to specific cues from the local microenvironment ( 3, 4), but they may also derive from other precursor cells, including resident mesenchymal progenitor cells, muscle satellite cells, and the recently found fibro/adipogenic progenitors (FAPs) ( 5).Ĭanonical effectors of fibrosis are the transforming growth factor–β (TGF-β) and wingless-related integration site (WNT) families of secreted ligands ( 6). Myofibroblasts acquire a spindle-shape or stellate morphology, are contractile and motile, express α–smooth muscle actin (αSMA), and secrete excess amounts of ECM components ( 3, 4). Activated fibroblasts or myofibroblasts are primarily responsible for the development and amplification of the fibrotic process ( 3, 4). In the case of chronic forms of tissue damage, increased production and deposition of the ECM result in a fibrotic disease that ultimately leads to organ failure ( 1, 3).
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Deposition and remodeling of the ECM occur physiologically during development and organ differentiation and, in the adult, during tissue regeneration or repair and wound healing following injury and inflammation ( 1). Connective tissue resident fibroblasts are the primary cells that produce and secrete the interstitial ECM, which defines the extent of tissue stiffness and architecture ( 1, 2). Mammalian tissues and organs preserve their structural and functional homeostasis by means of their supportive connective tissue, a three-dimensional network of cells and extracellular matrix (ECM) with different topological characteristics depending on the organ/tissue in which it is embedded ( 1). These findings reveal an unexpected exosome-mediated signaling pathway downstream of NEU1 deficiency that propagates a fibrotic disease and could be implicated in idiopathic forms of fibrosis in humans. Myofibroblast-derived exosomes fed to normal fibroblasts convert them into myofibroblasts, changing the recipient cells’ proliferative and migratory properties. These nanocarriers loaded with activated transforming growth factor–β and wingless-related integration site (WNT)/β-catenin signaling molecules propagate fibrotic signals to other cells, maintaining the tissue in a prolonged transitional status. We now report that in muscle connective tissue, Neu1 −/− fibroblasts have features of myofibroblasts and are proliferative, migratory, and exocytose large amounts of exosomes. Yet, the tissue-specific molecular consequences of this deregulated pathway are still unfolding. In Neu1 −/− mice, excessive lysosomal exocytosis is at the basis of disease pathogenesis. However, this can be automatically converted to compatible units via the pull-down menu.Lysosomal exocytosis is a ubiquitous process negatively regulated by neuraminidase 1 (NEU1), a sialidase mutated in the glycoprotein storage disease sialidosis.
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Triangle Volume (V): The volume is returned in cubic meters. INSTRUCTIONS: Choose units and enter the following: The Triangle Volume calculator computes the volume of a triangular Triangular Volume shaped object (such as a prism) given the length the triangle's three sides and the height (h) of the area.