UNIPROT #

P16234

PROTEIN NAME

PDGFR alpha

SYNONYMS

Alpha-type platelet-derived growth factor receptor antibody; CD140 antigen-like family member A antibody; CD140a antibody; CD140a antigen antibody; MGC74795 antibody; PDGF alpha chain antibody; PDGF-R-alpha antibody; PDGFR 2 antibody; PDGFR alpha antibody; PDGFR2 antibody; PDGFRA antibody; PDGFRA/BCR fusion antibody; PGFRA_HUMAN antibody; Platelet derived growth factor receptor 2 antibody; Platelet derived growth factor receptor alpha antibody; Platelet derived growth factor receptor alpha polypeptide antibody; Platelet derived growth factor receptor antibody; Rearranged in hypereosinophilia platelet derived growth factor receptor alpha fusion protein antibody; RHEPDGFRA antibody

SEQUENCE SIMILARITIES

Belongs to the protein kinase superfamily. Tyr protein kinase family. CSF-1/PDGF receptor subfamily.

TISSUE SPECIFICITY

Detected in platelets (at protein level). Widely expressed. Detected in brain, fibroblasts, smooth muscle, heart, and embryo. Expressed in primary and metastatic colon tumors and in normal colon tissue.

POST-TRANSLATIONAL MODIFICATION

N-glycosylated.; Ubiquitinated, leading to its internalization and degradation.; Autophosphorylated on tyrosine residues upon ligand binding. Autophosphorylation occurs in trans, i.e. one subunit of the dimeric receptor phosphorylates tyrosine residues on the other subunit. Phosphorylation at Tyr-731 and Tyr-742 is important for interaction with PIK3R1. Phosphorylation at Tyr-720 and Tyr-754 is important for interaction with PTPN11. Phosphorylation at Tyr-762 is important for interaction with CRK. Phosphorylation at Tyr-572 and Tyr-574 is important for interaction with SRC and SRC family members. Phosphorylation at Tyr-988 and Tyr-1018 is important for interaction with PLCG1.

SUBCELLULAR LOCATION

Golgi apparatus, Cell membrane, cilium.

FUNCTION

Platelet-derived growth factor (PDGF) is a mitogen for mesenchyme- and glia-derived cells. PDGF consists of two chains, A and B, which dimerize to form functionally distinct isoforms, PGDF-AA, PDGF-AB and PDGF-BB. These three isoforms bind with different affinities to two receptor types, PDGFR-α and -β, which are endowed with protein tyrosine kinase domains. PDGFR-α can bind to both A and B subunits of PDGF, while PDGFR-β can only bind the B subunit. Ligand binding promotes either homo- or heterodimerization of the PDGF receptors in a specific manner. PDGF-AA induces the dimerization of two α receptors, PDGF-AB induces dimerization of αα and αβ and PDGF-BB induces the formation of three types of dimers, αα, αβ and ββ. Translocation of the PDGFR-β gene with the Tel gene is linked to chronic myelomonocytic leukemia (CMML), a myelodysplastic syndrome, and demonstrates the oncogenic potential of the PDGF receptors.