platelet-derived growth factor C; PDGF-C; spinal cord-derived growth factor; SCDGF; fallotein; VEGF-E; contains: platelet-derived growth factor C, latent form; PDGFC latent form; contains: platelet-derived growth factor C, receptor-binding form; PDGFC receptor-binding form (PDGFC, SCDGF, UNQ174/PRO200)

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Function

potent mitogen & chemoattractant for cells of mesenchymal origin
binding of PDGFC to its affinity receptor elicits a variety of cellular responses.
appears to be involved in the 3 stages of wound healing: inflammation, proliferation & remodeling
role in fibrotic processes, in which transformation of interstitial fibroblasts into myofibroblasts plus collagen deposition occurs
acts as a specific ligand for PDGFR alpha homodimer, & alpha & beta heterodimer
binding to receptors induces their activation by Tyr phosphorylation
the CUB domain has mitogenic activity in coronary artery smooth muscle cells, suggesting a role beyond the maintenance of the latency of the PDGF domain
in the nucleus, PDGFC seems to have additional function
seems to be involved in palatogenesis (putative)
proteolytic removal of the N-terminal CUB domain releasing the core domain is necessary for unmasking the receptor- binding epitopes of the core domain
cleavage after basic residues in the hinge region (region connecting the CUB & growth factor domains) gives rise to the receptor-binding form
cleaved by PLAT & PLG
sumoylated by SUMO1
interacts (via CUB domain) with PLAT (via kringle domain)

expressed in the fallopian tube, vascular smooth muscle cells in kidney, breast & colon & in visceral smooth muscle of the gastrointestinal tract
highly expressed in retinal pigment epithelia
expressed in medulloblastoma
in the kidney, constitutively expressed in parietal epithelial cells of Bowman's capsule, tubular epithelial cells & in arterial endothelial cells (at protein level)
highly expressed in platelets, prostate, testis & uterus
weaker expression in the spleen, thymus, heart, pancreas, liver, ovary cells & small intestine
negligible expression in the colon & peripheral blood leukocytes
in the fetal kidney, detected in the developing mesangium, ureteric bud epithelium & the undifferentiated mesenchyme (at protein level)
up-regulated by EWS-FLI1 chimeric transcription factor in tumor derived cells
up-regulated in podocytes & interstitial cells after injury/activation of these cells
FGF2 activates PDGFC transcription via EGR1
up-regulated by TGFB1 in concert with FGF2

predominant PDGF isoform present in patients with proliferative vitreoretinopathy (PVR); plasmin is the major protease that processes PDGFC in the vitreous of PVR patients
expression increased in patients with uterine leiomyoma
downstream target of EWSR1 fusion proteins, contributing to the Ewing family tumors (EFT) malignant phenotype
the medulloblastoma phenotype is associated with PDGFR alpha expression & activation, with PDGFC as a major player in such endogenous autocrine loop
lower molecular weight form (around 43 kD) is present in patients with papillary thyroid carcinoma