A Novel Pathogenic Variant in NDP Gene With Incomplete Penetrance Manifests as X-Linked Familial Exudative Vitreoretinopathy

Ophthalmic Surg Lasers Imaging Retina. 2019 Feb 1;50(2):120-124. doi: 10.3928/23258160-20190129-10.

Abstract

Familial exudative vitreoretinopathy (FEVR) is a rare hereditary ocular disorder characterized by incomplete or abnormal development of peripheral retinal vasculature. The genes responsible for this disorder are associated with the wingless-related integration site (Wnt) signaling pathway, a critical pathway for the development of normal retinal vasculature. A pathogenic variant in any one of these genes may disrupt retinal vasculogenesis. Furthermore, the type and number of pathogenic variants may influence the severity of disease and clinical course. Here, the authors identify a novel pathogenic variant in the NDP gene, not previously described in the literature. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:120-124.].

Publication types

  • Case Reports

MeSH terms

  • Child, Preschool
  • Eye Diseases, Hereditary / genetics*
  • Eye Proteins / genetics*
  • Familial Exudative Vitreoretinopathies
  • Genetic Diseases, X-Linked*
  • Genetic Predisposition to Disease
  • Humans
  • Male
  • Mutation*
  • Nerve Tissue Proteins / genetics*
  • Pedigree
  • Penetrance
  • Retinal Diseases / genetics*

Substances

  • Eye Proteins
  • NDP protein, human
  • Nerve Tissue Proteins