Src family kinases negatively regulate platelet-derived growth factor alpha receptor-dependent signaling and disease progression

J Biol Chem. 2000 Mar 31;275(13):9620-7. doi: 10.1074/jbc.275.13.9620.

Abstract

We tested the hypothesis that Src family kinases (SFK) contribute to c-Cbl-mediated degradation of the platelet-derived growth factor (PDGF) alpha receptor (alphaPDGFR). Using either a receptor mutant that does not engage SFKs (F72/74), or cells that that lack SFKs, we found that SFKs contributed to degradation of the alphaPDGFR. Overexpression of c-Cbl also reduced the receptor half-life, but only if the receptor was able to engage SFKs. In cultured cells, prolonging the half-life of the receptor correlated with enhanced signaling and more efficient S phase entry, whereas accelerating receptor degradation had the opposite effect. Consistent with these tissue culture findings, there was a statistically significant increase in the onset of a proliferative retinal disease when animals were injected with cells expressing the F72/74 receptor, as compared with cells expressing the WT receptor. Our findings suggest that SFKs cooperate with c-Cbl to negatively regulate the alphaPDGFR, and that the SFK/c-Cbl suppression of alphaPDGFR output is relevant to the onset and progression of a proliferative disease.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • 3T3 Cells
  • Animals
  • DNA Replication
  • Disease Models, Animal
  • Disease Progression
  • Hydrolysis
  • Mice
  • Phosphorylation
  • Proto-Oncogene Proteins / metabolism
  • Proto-Oncogene Proteins c-cbl
  • Rabbits
  • Receptor, Platelet-Derived Growth Factor alpha / metabolism*
  • Signal Transduction*
  • Tyrosine / metabolism
  • Ubiquitin-Protein Ligases*
  • Vitreoretinopathy, Proliferative / enzymology
  • Vitreoretinopathy, Proliferative / metabolism*
  • src-Family Kinases / metabolism*

Substances

  • Proto-Oncogene Proteins
  • Tyrosine
  • Proto-Oncogene Proteins c-cbl
  • Ubiquitin-Protein Ligases
  • Receptor, Platelet-Derived Growth Factor alpha
  • src-Family Kinases
  • Cbl protein, mouse