ROBO2 gene variants in children with primary nonsyndromic vesicoureteral reflux with or without renal hypoplasia/dysplasia

Pediatr Res. 2016 Jul;80(1):72-6. doi: 10.1038/pr.2016.51. Epub 2016 Mar 22.

Abstract

Background: Primary nonsyndromic vesicoureteral reflux (VUR) and VUR with renal hypoplasia/dysplasia (VUR-RHD) are common congenital anomalies of the kidney and urinary tract (CAKUT). Sequence variations of the ROBO2 gene were investigated in children with nonsyndromic VUR or VUR-RHD.

Methods: Single-strand conformation polymorphism (SSCP) electrophoresis or multiple restriction fragment SSCP (MRF-SSCP), followed occasionally by direct sequencing, was used to screen 103 patients and 200 controls for nucleotide changes. Gene polymorphisms and transposable elements were investigated using bioinformatics.

Results: Two single-nucleotide polymorphisms were detected: IVS1-53 and IVS5-31. The frequency of A allele of IVS1-53G>A did not differ significantly between patients and controls. IVS1-53 does not affect mRNA splicing according to in silico analysis. IVS5-31A>G substitution was found in one patient, reported here for the first time in VUR. In silico results demonstrated alteration in two serine/arginine-rich (SR) protein-binding sites and two additional acceptor sites. The ROBO2 gene sequence was found to contain 25.9% transposable elements.

Conclusion: ROBO2 variants were not found to be associated with nonsyndromic VUR or VUR-RHD, providing further evidence for genetic heterogeneity. The role of transposable elements in ROBO2 gene expression in CAKUT needs further investigation since they are generally considered to be mutagens.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Alleles
  • Binding Sites
  • Case-Control Studies
  • Child, Preschool
  • Computational Biology
  • DNA Transposable Elements
  • Female
  • Gene Frequency
  • Genetic Predisposition to Disease
  • Genotype
  • Humans
  • Infant
  • Kidney Diseases / complications
  • Kidney Diseases / genetics*
  • Male
  • Nucleotides / chemistry
  • Polymerase Chain Reaction
  • Polymorphism, Single Nucleotide*
  • Polymorphism, Single-Stranded Conformational
  • Receptors, Immunologic / genetics*
  • Sequence Analysis, DNA
  • Vesico-Ureteral Reflux / complications
  • Vesico-Ureteral Reflux / genetics*

Substances

  • DNA Transposable Elements
  • Nucleotides
  • ROBO2 protein, human
  • Receptors, Immunologic