Platelet Derived Growth Factor AA, Recombinant, Mouse (PDGF-AA, Platelet-Derived Growth Factor Subunit A, PDGF Subunit A, Platelet-Derived Growth Factor A Chain, Platelet-Derived Growth Factor alpha Polypeptide, PDGF-1, PDGFA, PDGF1)

The protein encoded by this gene is a member of the platelet-derived growth factor family. The four members of this family are mitogenic factors for cells of mesenchymal origin and are characterized by a motif of eight cysteines. This gene product can exist either as a homodimer (PDGF-BB) or as a heterodimer with the platelet-derived growth factor alpha polypeptide (PDGF-AB), where the dimers are connected by disulfide bonds. Mutations in this gene are associated with meningioma. Reciprocal translocations between chromosomes 22 and 17, at sites where this gene and that for collagen type 1, alpha 1 are located, are associated with a particular type of skin tumor called dermatofibrosarcoma protuberans resulting from unregulated expression of growth factor. Two alternatively spliced transcript variants encoding different isoforms have been identified for this gene. [provided by RefSeq, Feb 2013]

PDGFB: Growth factor that plays an essential role in the regulation of embryonic development, cell proliferation, cell migration, survival and chemotaxis. Potent mitogen for cells of mesenchymal origin. Required for normal proliferation and recruitment of pericytes and vascular smooth muscle cells in the central nervous system, skin, lung, heart and placenta. Required for normal blood vessel development, and for normal development of kidney glomeruli. Plays an important role in wound healing. Signaling is modulated by the formation of heterodimers with PDGFA. A chromosomal aberration involving PDGFB is found in dermatofibrosarcoma protuberans. Translocation t(17;22)(q22;q13) with PDGFB. Belongs to the PDGF/VEGF growth factor family.

Protein type: Secreted, signal peptide; Motility/polarity/chemotaxis; Secreted; Oncoprotein

Chromosomal Location of Human Ortholog: 22q13.1

Cellular Component: Golgi membrane; extracellular space; cell surface; basolateral plasma membrane; endoplasmic reticulum lumen; cytoplasm; extracellular region

Molecular Function: collagen binding; identical protein binding; protein binding; protein homodimerization activity; growth factor activity; protein heterodimerization activity; platelet-derived growth factor binding; platelet-derived growth factor receptor binding; superoxide-generating NADPH oxidase activator activity; chemoattractant activity

Biological Process: extracellular matrix organization and biogenesis; positive regulation of cyclin-dependent protein kinase activity; nerve growth factor receptor signaling pathway; heart development; positive regulation of transcription, DNA-dependent; positive regulation of fibroblast growth factor receptor signaling pathway; cell projection biogenesis; protein amino acid phosphorylation; monocyte chemotaxis; positive regulation of MAP kinase activity; positive regulation of fibroblast proliferation; positive chemotaxis; transforming growth factor beta receptor signaling pathway; cell growth; positive regulation of mitotic cell cycle, embryonic; substrate-bound cell migration; response to drug; platelet activation; fibroblast growth factor receptor signaling pathway; positive regulation of mitosis; positive regulation of chemotaxis; activation of protein kinase B; positive regulation of blood vessel endothelial cell migration; positive regulation of protein amino acid autophosphorylation; positive regulation of phosphoinositide 3-kinase cascade; positive regulation of peptidyl-tyrosine phosphorylation; activation of protein kinase activity; blood vessel morphogenesis; positive regulation of endothelial cell proliferation; negative regulation of transcription, DNA-dependent; actin cytoskeleton organization and biogenesis; embryonic placenta development; peptidyl-tyrosine phosphorylation; positive regulation of smooth muscle cell proliferation; platelet-derived growth factor receptor signaling pathway; positive regulation of smooth muscle cell migration; response to estradiol stimulus; response to insulin stimulus; platelet degranulation; positive regulation of MAPKKK cascade; positive regulation of glomerular filtration; positive regulation of cell proliferation; response to wounding; hemopoiesis; DNA replication; negative regulation of cell migration; epidermal growth factor receptor signaling pathway; phosphoinositide-mediated signaling; negative regulation of phosphatidylinositol biosynthetic process; positive regulation of phosphoinositide 3-kinase activity; peptidyl-serine phosphorylation; response to hypoxia; innate immune response; blood coagulation; positive regulation of DNA replication; positive regulation of cell migration

Disease: Meningioma, Familial, Susceptibility To; Dermatofibrosarcoma Protuberans; Basal Ganglia Calcification, Idiopathic, 5; Basal Ganglia Calcification, Idiopathic, 1