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Beschreibung

Platelet-derived growth factor subunit B (UniProt: P01127, also known as PDGF subunit B, PDGF-2, Platelet-derived growth factor B chain, Platelet-derived growth factor beta polypeptide, Proto-oncogene c-Sis, Becaplermin) is encoded by the PDGFB (also known as PDGF2, SIS) gene (Gene ID: 5155) in human. PDGFs are a family of four cystine-knot-type growth factors (PDGF-A, -B, -C and -D) that are dimeric mitogenic signaling molecules. In the early developmental stage, they drive the proliferation of undifferentiated mesenchyme and some progenitor populations. During later maturation stages they participate in tissue remodeling, cellular differentiation, and morphogenesis. PDGF A and B can exist as two A subunits (PDGF-AA), two B subunits (PDGF-BB), or one of each (PDGF-AB). These dimeric forms are differentially expressed in various cell types. Their action is mediated via two types of receptors PDGFR and PDGFRbeta, which belong to the class III receptor tyrosine kinases and exhibit different expression patterns and physiological roles. PDGFR signaling controls gastrulation and the development of several organs such as lung, intestine, skin, testis, kidney, bones, and neuroprotective tissues and PDGFRbeta signaling is an essential regulator of early hematopoiesis and blood vessel formation. PDGF-BB is synthesized with a signal peptide (aa 1-20) and two propeptides (aa 21-81 and 191-241), which are cleaved off to generate the active form (aa 82-190). PDGF-BB is expressed at high levels in the heart, placenta, fetal kidney, and substantia nigra in the brain and at moderate levels in hippocampus, skeletal muscle, kidney, and lung. It is required for normal blood vessel development, and for normal development of kidney glomeruli and is shown to plays an important role in wound healing. It is released by platelets upon wounding. Mutations in PDGFB gene have been linked to idiopathic basal ganglia calcification that leads to a wide spectrum of neuropsychiatric symptoms, including Parkinsonism, dystonia tremor, ataxia, and dementia. (Ref.: Chen, PH., et al. (2013). Biochim. Biophys. Acta. 1834(10), 2176-2186).