FAK-Src-paxillin system expression and disease outcome in human neuroblastoma

Pediatr Hematol Oncol. 2017 May;34(4):221-230. doi: 10.1080/08880018.2017.1360969. Epub 2017 Oct 17.


Background: Neuroblastoma (NB) often presents with metastatic disease and poor survival. The need for new prognostic markers remains invaluable. The FAK-Src-Paxillin protein system is associated with aggressive phenotype in adult malignancies but is largely unexplored in pediatric NB.

Objective: To assess FAK-Src-Paxillin protein expression in human NB cell lines and clinical cytology material and to delineate its association with survival.

Design/methods: Western blot and immunohistochemistry were applied for FAK-Src-Paxillin expression in NB cell lines and 23 human cytology specimens, respectively. Protein expression in human clinical samples was correlated with clinicopathological parameters, MYCN amplification and survival.

Results: FAK, Src and Paxillin proteins are expressed in human NB cells lines, and can be detected in clinical cytology specimens from NB patients, (59%, 32% and 33% respectively). Simultaneous FAK-Src-Paxillin expression was noted in 30% of NB patients. Children with concomitant positivity FAK, Src, and Paxillin tumors, as well as MYCN amplification, had increased mortality compared to those without.

Conclusions: FAK-Src-Paxillin system is a marker of unfavorable prognosis for human NB patients but also a promising therapeutic target.

Keywords: FAK; NMYC; Src; neuroblastoma; paxillin.

MeSH terms

  • Animals
  • Biomarkers, Tumor / biosynthesis*
  • Child
  • Child, Preschool
  • Disease-Free Survival
  • Female
  • Focal Adhesion Kinase 1 / biosynthesis*
  • Gene Expression Regulation, Neoplastic*
  • Humans
  • Infant
  • K562 Cells
  • Male
  • Mice
  • N-Myc Proto-Oncogene Protein / biosynthesis
  • NIH 3T3 Cells
  • Neuroblastoma* / metabolism
  • Neuroblastoma* / mortality
  • Neuroblastoma* / pathology
  • Paxillin / biosynthesis*
  • Proto-Oncogene Proteins pp60(c-src) / biosynthesis*
  • Survival Rate


  • Biomarkers, Tumor
  • MYCN protein, human
  • N-Myc Proto-Oncogene Protein
  • PXN protein, human
  • Paxillin
  • Focal Adhesion Kinase 1
  • PTK2 protein, human
  • Proto-Oncogene Proteins pp60(c-src)