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Fibrodysplasia ossificans progressiva (/ ˌ f aɪ b r oʊ d ɪ ˈ s p l eɪ ʒ (i) ə ɒ ˈ s ɪ f ɪ k æ n z p r ə ˈ ɡ r ɛ s ɪ v ə /; [1] abbr. FOP), also called Münchmeyer disease or formerly myositis ossificans progressiva, is an extremely rare connective tissue disease in which fibrous connective tissue such as muscle, tendons, and ligaments turn into bone tissue (ossification).
Fibroblasts within the muscle deposit scar tissue, which can impair muscle function, and is a significant part of the pathology of muscular dystrophies. Satellite cells proliferate following muscle trauma [ 11 ] and form new myofibers through a process similar to fetal muscle development. [ 12 ]
Myostatin is a myokine that is produced and released by myocytes and acts on muscle cells to inhibit muscle growth. [7] Myostatin is a secreted growth differentiation factor that is a member of the TGF beta protein family. [8] [9] Myostatin is assembled and produced in skeletal muscle before it is released into the blood stream. [10]
A bone growth factor is a growth factor that stimulates the growth of bone tissue. [1] [2]Known bone growth factors include insulin-like growth factor-1 (IGF-1), insulin-like growth factor-2 (IGF-2), transforming growth factor beta (TGF-β), fibroblast growth factors (FGFs), platelet-derived growth factor (PDGF), parathyroid hormone-related peptide (PTHrP), bone morphogenetic proteins (BMPs ...
Bone pain is a common complication of fibrous dysplasia. It may present at any age, but most commonly develops during adolescence and progresses into adulthood. [7] Bone marrow stromal cells in fibrous dysplasia produce excess amounts of the phosphate-regulating hormone fibroblast growth factor-23 (FGF23), leading to loss of phosphate in the ...
Fibroblast growth factor receptor 3 (FGFR-3) is a protein that in humans is encoded by the FGFR3 gene. [5] FGFR3 has also been designated as CD333 ( cluster of differentiation 333). The gene, which is located on chromosome 4 , location p16.3, is expressed in tissues such as the cartilage, brain, intestine, and kidneys.
Bone Modeling is known as formation of new bone from either cartilage or by direct deposition, mostly during growth and development. This usually does lead to changes in size and shape over time. [3] Growth Sites is a term proposed by Baume. [4] Growth Sites serve as a location in the bone where the actual growth occurs.
Bone morphogenetic proteins (BMPs) are a group of growth factors also known as cytokines and as metabologens. [1] Professor Marshall Urist and Professor Hari Reddi discovered their ability to induce the formation of bone and cartilage, BMPs are now considered to constitute a group of pivotal morphogenetic signals, orchestrating tissue architecture throughout the body.