2 Matching Annotations
  1. Aug 2026
    1. 92 RHO c.180C>A p.Tyr60* – [a] ROM1: f 31 RP ad no Ger c.178C>A p.Pro60Thr; – [42], [43] c.323C>T p.Thr108Met rs146358003 [42], [43] ABCA4: c.1654G>A p.Val552Ile rs145525174 [44] c.5714+5G>A RP2: splice rs61751407 [45] c.844C>T RPGRIP1: c.2510C>G p.Arg282Trp rs1805147 [17], [18] p.Ala837Gly – [22]

      Case#: Eisenberger Patient 92, female, 31yo, German

      DiseaseAssertion: Autosomal dominant RP

      FamilyInfo: non-consanguineous

      CasePresentingHPOs:

      CaseHPOFreeText: "The diagnoses of all patients were established by medical history, family history and detailed clinical evaluation of vision. Ophthalmological examination included stereoscopic funduscopy, standard ERG, perimetry, measurement of dark adaptation, and determination of best-corrected visual acuity in most patients."

      CaseNotHPOs:

      CaseNotHPOFreeText:

      GenotypingMethod: NGS for the exons of 55 RP and LCA genes, Sanger for confirmation

      PreviouslyPublished: n/a

      Variant: Allele 1: RHO c.180C>A p.Tyr60; Allele 2*: ROM1: c.178C>A p.Pro60Thr; c.323C>T p.Thr108Met;

      ABCA4: c.1654G>A p.Val552Ile; c.5714+5G>A; <br /> RP2: c.844C>T p.Arg282Trp; <br /> RPGRIP1: c.2510C>G p.Ala837Gly

      ClinVar:

      CAID: CA239745

      SupplementalData: n/a

  2. Apr 2022
    1. The DICER1 syndrome is an autosomal dominant tumor‐predisposition disorder

      Gene: DICER1 PMID: 30672147 HGNCID: Not found Inheritance Pattern: autosomal dominant Disease Entity: Pleuropulmonary Blastoma, Cystic Nephroma, Sertoli-Leydig tumors, Multinodular goiter, thyroid cancer, rhabdomysarcoma, pineoblastoma Mutation: germline Zygosity: not stated Variant: p.Asp1810Asn, c.4206+1G>C [splice],p.Gly1824Val, p.Ser1160Tyr, p.Ala1578Thr, p.Leu1469Pro, p.Ser1160Tyr, p.Ile528Thr, p.Pro1836Leu, p.Glu904, p.Tyr1835Ser, p.Ile528Thr, p.Arg1342His, p.Phe1650Cys, p.Trp1481Arg, p.Arg201His, p.Asp1390His, p.Asp1810Asn c.4206+1G>C, p.Trp1397Arg, p.Ala1578Thr Family Info: none provided