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Gene Review

PDGFRB  -  platelet-derived growth factor receptor,...

Homo sapiens

Synonyms: Beta platelet-derived growth factor receptor, Beta-type platelet-derived growth factor receptor, CD140 antigen-like family member B, CD140B, CD140b, ...
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Disease relevance of PDGFRB

  • Similarly, the drug has now been shown to display equally impressive therapeutic activity in eosinophilia-associated chronic myeloproliferative disorders that are characterized by activating mutations of either the PDGFRB or the PDGFRA gene [1].
  • The TEL-PDGFRB fusion oncogene is associated with chronic myelomonocytic leukemia (CMML) and results in the expression of a constitutively active tyrosine kinase [2].
  • At the center of these new developments are mutations involving the platelet-derived growth factor receptor genes (PDGFRA and PDGFRB), which have been pathogenetically linked to clonal eosinophilia, and their presence predicts complete as well as durable treatment responses to imatinib mesylate [3].
  • The fourth patient had leukocytosis, eosinophilia, and a t(5;12) translocation involving PDGFRB and an unknown partner gene; he also had extensive raised, ulcerated skin lesions that had been present for a long time [4].
  • Coexpression of platelet-derived growth factor (PDGF) and PDGF-receptor genes by primary human astrocytomas may contribute to their development and maintenance [5].

Psychiatry related information on PDGFRB


High impact information on PDGFRB


Chemical compound and disease context of PDGFRB

  • For example, the presence of either PDGFRA or PDGFRB mutations warrants the use of imatinib in clonal eosinophilia [11].
  • SU101 inhibited PDGF-stimulated tyrosine phosphorylation of PDGF receptor (PDGFR) beta in C6 (rat glioma) and NIH3T3 cells engineered to overexpress human PDGFRbeta (3T3-PDGFRbeta) [12].
  • The expression of platelet derived growth factors (PDGF), the PDGF-Receptor (R) (alpha and beta types), epidermal growth factor (EGF)-Receptor (R) and sex hormone (oestrogen and progesterone) receptors was studied in 22 meningiomas [13].
  • Using both Swiss 3T3 and human glioma U-1242MG cells, western blots with anti-PDGFR and anti-phosphotyrosine antibodies confirmed that these bands were the PDGFR monomer and dimer, respectively, and that phosphotyrosine was present in these bands only after cells were stimulated with PDGF [14].
  • We found that TS model cells derived from tuberin heterozygous mice and from a human renal angiomyolipoma are highly sensitive to PDGFR antagonists and that these cells express PDGFRbeta [15].

Biological context of PDGFRB

  • Translocation t(5;12)(q33;p13), resulting in an ETV6/PDGFRB gene fusion, is a recurrent chromosomal abnormality associated with chronic myelomonocytic leukemia (CMML) [16].
  • In four selected genes with known role in the cell cycle regulation (JunD, ACK1, PDGFRB, and TCF4), we confirmed expression changes after HDACi by quantitative analysis [17].
  • In order to elucidate the control mechanism for the receptor expression, we have isolated an enhancer from two P1 clones that together contain a 102 kb NotI region covering the entire human PDGFRB gene [18].
  • Most cases have a normal or aneuploid karyotype, but a minority present with a reciprocal translocation that disrupts specific tyrosine kinase genes, most commonly PDGFRB or FGFR1 [19].
  • Six patients showed a pattern of one fused signal (from the normal allele) and one pair of signals separated by more than one signal width in > 85% of interphase cells, indicating that PDGFRB was disrupted [20].

Anatomical context of PDGFRB

  • To test the hypothesis that activating RTK mutations cooperate with AE to cause leukemia, we transplanted retrovirally transduced murine bone marrow coexpressing TEL-PDGFRB and AE into lethally irradiated syngeneic mice [21].
  • Imatinib mesylate is a small molecule drug that in vitro inhibits the Abelson (Abl), Arg (abl-related gene), stem cell factor receptor (Kit), and platelet-derived growth factor receptor A and B (PDGFRA and PDGFRB) tyrosine kinases [1].
  • AF tumors expressed minimal to null levels of KIT and PDGFRA but expressed levels of PDGFRB that are comparable with normal fibroblasts [22].
  • When the new gene was transfected into COS-1 cells, a characteristic pattern of binding of the PDGF isoforms was observed, which was different from the pattern observed with the known PDGF receptor [23].
  • Incubation of lysates prepared from quiescent mesangial cells with purified PDGFR resulted in STAT1alpha activation [24].

Associations of PDGFRB with chemical compounds

  • Complete remission of TEL-PDGFRB-induced myeloproliferative disease in mice by receptor tyrosine kinase inhibitor SU11657 [2].
  • In the 29 patients without FIP1L1-PDGFRA, no activating mutation of PDGFRA/PDGFRB was detected; however; one patient responded to imatinib [25].
  • An accurate molecular diagnosis of PDGFRB-rearranged patients has become increasingly important since recent data have indicated that they respond very well to imatinib mesylate therapy [20].
  • Mutation of Tyr-751 to Phe or Gly, or mutation of the catalytic domain to abolish kinase activity, blocked association of the PDGF-R with the PI kinase and the three proteins [26].
  • A PDGFR mutant (F5) that lacks high-affinity binding sites for the Src homology 2 domain-containing proteins phosphatidylinositol-3 kinase (PI-3 kinase), Ras guanosine triphosphatase activating protein, phospholipase C-gamma, and a phosphotyrosine phosphatase (Syp) remained at the cell periphery [27].

Physical interactions of PDGFRB


Enzymatic interactions of PDGFRB


Co-localisations of PDGFRB

  • Double-label assays showed that PDGFR beta was often colocalized with each PDGF ligand, especially in pigmented cells [36].

Regulatory relationships of PDGFRB


Other interactions of PDGFRB


Analytical, diagnostic and therapeutic context of PDGFRB


  1. Imatinib targets other than bcr/abl and their clinical relevance in myeloid disorders. Pardanani, A., Tefferi, A. Blood (2004) [Pubmed]
  2. Complete remission of TEL-PDGFRB-induced myeloproliferative disease in mice by receptor tyrosine kinase inhibitor SU11657. Cain, J.A., Grisolano, J.L., Laird, A.D., Tomasson, M.H. Blood (2004) [Pubmed]
  3. Imatinib therapy in clonal eosinophilic disorders, including systemic mastocytosis. Tefferi, A., Pardanani, A. Int. J. Hematol. (2004) [Pubmed]
  4. Response to imatinib mesylate in patients with chronic myeloproliferative diseases with rearrangements of the platelet-derived growth factor receptor beta. Apperley, J.F., Gardembas, M., Melo, J.V., Russell-Jones, R., Bain, B.J., Baxter, E.J., Chase, A., Chessells, J.M., Colombat, M., Dearden, C.E., Dimitrijevic, S., Mahon, F.X., Marin, D., Nikolova, Z., Olavarria, E., Silberman, S., Schultheis, B., Cross, N.C., Goldman, J.M. N. Engl. J. Med. (2002) [Pubmed]
  5. Coexpression of platelet-derived growth factor (PDGF) and PDGF-receptor genes by primary human astrocytomas may contribute to their development and maintenance. Maxwell, M., Naber, S.P., Wolfe, H.J., Galanopoulos, T., Hedley-Whyte, E.T., Black, P.M., Antoniades, H.N. J. Clin. Invest. (1990) [Pubmed]
  6. Platelet-derived growth factor (PDGF) and PDGF receptors in rat corpus cavernosum: changes in expression after transient in vivo hypoxia. Aversa, A., Basciani, S., Visca, P., Arizzi, M., Gnessi, L., Frajese, G., Fabbri, A. J. Endocrinol. (2001) [Pubmed]
  7. PDGFRB is overexpressed in metastatic medulloblastoma. Gilbertson, R.J., Clifford, S.C. Nat. Genet. (2003) [Pubmed]
  8. Fusion of PDGF receptor beta to a novel ets-like gene, tel, in chronic myelomonocytic leukemia with t(5;12) chromosomal translocation. Golub, T.R., Barker, G.F., Lovett, M., Gilliland, D.G. Cell (1994) [Pubmed]
  9. Phospholipase C-gamma 1 and phosphatidylinositol 3 kinase are the downstream mediators of the PDGF receptor's mitogenic signal. Valius, M., Kazlauskas, A. Cell (1993) [Pubmed]
  10. Association between the PDGF receptor and members of the src family of tyrosine kinases. Kypta, R.M., Goldberg, Y., Ulug, E.T., Courtneidge, S.A. Cell (1990) [Pubmed]
  11. Modern diagnosis and treatment of primary eosinophilia. Tefferi, A. Acta Haematol. (2005) [Pubmed]
  12. Inhibition of platelet-derived growth factor-mediated signal transduction and tumor growth by N-[4-(trifluoromethyl)-phenyl]5-methylisoxazole-4-carboxamide. Shawver, L.K., Schwartz, D.P., Mann, E., Chen, H., Tsai, J., Chu, L., Taylorson, L., Longhi, M., Meredith, S., Germain, L., Jacobs, J.S., Tang, C., Ullrich, A., Berens, M.E., Hersh, E., McMahon, G., Hirth, K.P., Powell, T.J. Clin. Cancer Res. (1997) [Pubmed]
  13. Expression of PDGF, PDGF-receptor, EGF-receptor and sex hormone receptors on meningioma. Kuratsu, J.I., Seto, H., Kochi, M., Ushio, Y. Acta neurochirurgica. (1994) [Pubmed]
  14. Gangliosides inhibit platelet-derived growth factor-stimulated receptor dimerization in human glioma U-1242MG and Swiss 3T3 cells. Van Brocklyn, J., Bremer, E.G., Yates, A.J. J. Neurochem. (1993) [Pubmed]
  15. Functional tyrosine kinase inhibitor profiling: a generally applicable method points to a novel role of platelet-derived growth factor receptor-beta in tuberous sclerosis. Arbiser, J.L., Govindarajan, B., Bai, X., Onda, H., Kazlauskas, A., Lim, S.D., Amin, M.B., Claesson-Welsh, L. Am. J. Pathol. (2002) [Pubmed]
  16. A new subtype of pre-B acute lymphoblastic leukemia with t(5;12)(q31q33;p12), molecularly and cytogenetically distinct from t(5;12) in chronic myelomonocytic leukemia. Wlodarska, I., Aventín, A., Inglés-Esteve, J., Falzetti, D., Criel, A., Cassiman, J.J., Mecucci, C., Van den Berghe, H., Marynen, P. Blood (1997) [Pubmed]
  17. The identification of (ETV6)/RUNX1-regulated genes in lymphopoiesis using histone deacetylase inhibitors in ETV6/RUNX1-positive lymphoid leukemic cells. Starkova, J., Madzo, J., Cario, G., Kalina, T., Ford, A., Zaliova, M., Hrusak, O., Trka, J. Clin. Cancer Res. (2007) [Pubmed]
  18. Activity of a novel PDGF beta-receptor enhancer during the cell cycle and upon differentiation of neuroblastoma. Kaneko, M., Yang, W., Matsumoto, Y., Watt, F., Funa, K. Exp. Cell Res. (2006) [Pubmed]
  19. Tyrosine kinase fusion genes in chronic myeloproliferative diseases. Cross, N.C., Reiter, A. Leukemia (2002) [Pubmed]
  20. Novel translocations that disrupt the platelet-derived growth factor receptor beta (PDGFRB) gene in BCR-ABL-negative chronic myeloproliferative disorders. Baxter, E.J., Kulkarni, S., Vizmanos, J.L., Jaju, R., Martinelli, G., Testoni, N., Hughes, G., Salamanchuk, Z., Calasanz, M.J., Lahortiga, I., Pocock, C.F., Dang, R., Fidler, C., Wainscoat, J.S., Boultwood, J., Cross, N.C. Br. J. Haematol. (2003) [Pubmed]
  21. An activated receptor tyrosine kinase, TEL/PDGFbetaR, cooperates with AML1/ETO to induce acute myeloid leukemia in mice. Grisolano, J.L., O'Neal, J., Cain, J., Tomasson, M.H. Proc. Natl. Acad. Sci. U.S.A. (2003) [Pubmed]
  22. Clinical and molecular studies of the effect of imatinib on advanced aggressive fibromatosis (desmoid tumor). Heinrich, M.C., McArthur, G.A., Demetri, G.D., Joensuu, H., Bono, P., Herrmann, R., Hirte, H., Cresta, S., Koslin, D.B., Corless, C.L., Dirnhofer, S., van Oosterom, A.T., Nikolova, Z., Dimitrijevic, S., Fletcher, J.A. J. Clin. Oncol. (2006) [Pubmed]
  23. Isolation of a novel receptor cDNA establishes the existence of two PDGF receptor genes. Matsui, T., Heidaran, M., Miki, T., Popescu, N., La Rochelle, W., Kraus, M., Pierce, J., Aaronson, S. Science (1989) [Pubmed]
  24. Association and direct activation of signal transducer and activator of transcription1alpha by platelet-derived growth factor receptor. Choudhury, G.G., Ghosh-Choudhury, N., Abboud, H.E. J. Clin. Invest. (1998) [Pubmed]
  25. Molecular characterization of the idiopathic hypereosinophilic syndrome (HES) in 35 French patients with normal conventional cytogenetics. Roche-Lestienne, C., Lepers, S., Soenen-Cornu, V., Kahn, J.E., Laï, J.L., Hachulla, E., Drupt, F., Demarty, A.L., Roumier, A.S., Gardembas, M., Dib, M., Philippe, N., Cambier, N., Barete, S., Libersa, C., Bletry, O., Hatron, P.Y., Quesnel, B., Rose, C., Maloum, K., Blanchet, O., Fenaux, P., Prin, L., Preudhomme, C. Leukemia (2005) [Pubmed]
  26. Autophosphorylation of the PDGF receptor in the kinase insert region regulates interactions with cell proteins. Kazlauskas, A., Cooper, J.A. Cell (1989) [Pubmed]
  27. Disruption of PDGF receptor trafficking by mutation of its PI-3 kinase binding sites. Joly, M., Kazlauskas, A., Fay, F.S., Corvera, S. Science (1994) [Pubmed]
  28. A new function for a phosphotyrosine phosphatase: linking GRB2-Sos to a receptor tyrosine kinase. Li, W., Nishimura, R., Kashishian, A., Batzer, A.G., Kim, W.J., Cooper, J.A., Schlessinger, J. Mol. Cell. Biol. (1994) [Pubmed]
  29. Heparin-like glycosaminoglycans influence growth and phenotype of human arterial smooth muscle cells in vitro. II. The platelet-derived growth factor A-chain contains a sequence that specifically binds heparin. Fager, G., Camejo, G., Olsson, U., Ostergren-Lundén, G., Bondjers, G. In Vitro Cell. Dev. Biol. (1992) [Pubmed]
  30. SHP-2 is involved in heterodimer specific loss of phosphorylation of Tyr771 in the PDGF beta-receptor. Ekman, S., Kallin, A., Engström, U., Heldin, C.H., Rönnstrand, L. Oncogene (2002) [Pubmed]
  31. APS, an adaptor protein containing PH and SH2 domains, is associated with the PDGF receptor and c-Cbl and inhibits PDGF-induced mitogenesis. Yokouchi, M., Wakioka, T., Sakamoto, H., Yasukawa, H., Ohtsuka, S., Sasaki, A., Ohtsubo, M., Valius, M., Inoue, A., Komiya, S., Yoshimura, A. Oncogene (1999) [Pubmed]
  32. Formation of signal transfer complexes between stem cell and platelet-derived growth factor receptors and SH2 domain proteins in vitro. Herbst, R., Shearman, M.S., Jallal, B., Schlessinger, J., Ullrich, A. Biochemistry (1995) [Pubmed]
  33. Activation of a phosphotyrosine phosphatase by tyrosine phosphorylation. Vogel, W., Lammers, R., Huang, J., Ullrich, A. Science (1993) [Pubmed]
  34. Association of Fyn with the activated platelet-derived growth factor receptor: requirements for binding and phosphorylation. Twamley, G.M., Kypta, R.M., Hall, B., Courtneidge, S.A. Oncogene (1992) [Pubmed]
  35. Inactivation of platelet-derived growth factor receptor by the tumor suppressor PTEN provides a novel mechanism of action of the phosphatase. Mahimainathan, L., Choudhury, G.G. J. Biol. Chem. (2004) [Pubmed]
  36. Platelet-derived growth factor ligands and receptors immunolocalized in proliferative retinal diseases. Robbins, S.G., Mixon, R.N., Wilson, D.J., Hart, C.E., Robertson, J.E., Westra, I., Planck, S.R., Rosenbaum, J.T. Invest. Ophthalmol. Vis. Sci. (1994) [Pubmed]
  37. Inhibition of growth factor-mediated tyrosine phosphorylation in vascular smooth muscle by PD 089828, a new synthetic protein tyrosine kinase inhibitor. Dahring, T.K., Lu, G.H., Hamby, J.M., Batley, B.L., Kraker, A.J., Panek, R.L. J. Pharmacol. Exp. Ther. (1997) [Pubmed]
  38. Hematopoietic tissues, as a playground of receptor tyrosine kinases of the PDGF-receptor family. Yoshida, H., Takakura, N., Hirashima, M., Kataoka, H., Tsuchida, K., Nishikawa, S., Nishikawa, S. Dev. Comp. Immunol. (1998) [Pubmed]
  39. Overexpression of the NF2 gene inhibits schwannoma cell proliferation through promoting PDGFR degradation. Fraenzer, J.T., Pan, H., Minimo, L., Smith, G.M., Knauer, D., Hung, G. Int. J. Oncol. (2003) [Pubmed]
  40. Basic fibroblast growth factor induces expression of the PDGF receptor-alpha on human bronchial smooth muscle cells. Bonner, J.C., Badgett, A., Lindroos, P.M., Coin, P.G. Am. J. Physiol. (1996) [Pubmed]
  41. Tyrosines 1021 and 1009 are phosphorylation sites in the carboxy terminus of the platelet-derived growth factor receptor beta subunit and are required for binding of phospholipase C gamma and a 64-kilodalton protein, respectively. Valius, M., Bazenet, C., Kazlauskas, A. Mol. Cell. Biol. (1993) [Pubmed]
  42. Tandem linkage of human CSF-1 receptor (c-fms) and PDGF receptor genes. Roberts, W.M., Look, A.T., Roussel, M.F., Sherr, C.J. Cell (1988) [Pubmed]
  43. A human osteosarcoma cell line secretes a growth factor structurally related to a homodimer of PDGF A-chains. Heldin, C.H., Johnsson, A., Wennergren, S., Wernstedt, C., Betsholtz, C., Westermark, B. Nature (1986) [Pubmed]
  44. Urokinase-induced signaling in human vascular smooth muscle cells is mediated by PDGFR-beta. Kiyan, J., Kiyan, R., Haller, H., Dumler, I. EMBO J. (2005) [Pubmed]
  45. Synergistic roles of platelet-derived growth factor-BB and interleukin-1beta in phenotypic modulation of human aortic smooth muscle cells. Chen, C.N., Li, Y.S., Yeh, Y.T., Lee, P.L., Usami, S., Chien, S., Chiu, J.J. Proc. Natl. Acad. Sci. U.S.A. (2006) [Pubmed]
  46. Platelet-derived growth factor (PDGF) autocrine signaling regulates survival and mitogenic pathways in glioblastoma cells: evidence that the novel PDGF-C and PDGF-D ligands may play a role in the development of brain tumors. Lokker, N.A., Sullivan, C.M., Hollenbach, S.J., Israel, M.A., Giese, N.A. Cancer Res. (2002) [Pubmed]
  47. Platelet-derived growth factor C (PDGF-C), a novel growth factor that binds to PDGF alpha and beta receptor. Gilbertson, D.G., Duff, M.E., West, J.W., Kelly, J.D., Sheppard, P.O., Hofstrand, P.D., Gao, Z., Shoemaker, K., Bukowski, T.R., Moore, M., Feldhaus, A.L., Humes, J.M., Palmer, T.E., Hart, C.E. J. Biol. Chem. (2001) [Pubmed]
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