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PRPH2

MIM.179605 6p21.1-cen

Definition: PRPH2 and ROM1 are tetraspanning membrane proteins that assemble into noncovalent tetramers and higher order disulfide-linked oligomers and are involved in photoreceptor disc morphogenesis.

Pathology

- germline mutations in

  • autosomal dominant retinitis pigmentosa locus 7 (RP7) (MIM.608133)
  • retinitis punctata albescens (MIM.136880)
  • macular dystrophy
  • adult vitelliform macular dystrophy (MIM.608161)
  • butterfly dystrophy of the retinal pigment epithelium (MIM.169150)
  • retinitis pigmentosa with bull’s-eye maculopathy (MIM.153870)
  • adult-onset vitelliform macular dystrophy (AVMD) (MIM.608161)
  • patterned dystrophy of the retina (MIM.169150)
  • fundus flavimaculatus (MIM.248200)

See also

- PRPHs

  • PRPH1