PABPN1

poly(A) binding protein nuclear 1
OMIM: 602279, Gene2Phenotype

4 panels

Panel Reviews Mode of inheritance Details
4 panels
Red PABPN1 in Congenital muscular dystrophy


Level 2: Neurology
Version 6.8
Latest signed off version: v6.0 (30 Apr 2025)

Component of the following Super Panels:

  • Hypotonic infant
  • Other rare neuromuscular disorders
  • review BOTH monoallelic and biallelic, autosomal or pseudoautosomal
    Sources
    • Expert Review Red
    • Emory Genetics Laboratory
    • Radboud University Medical Center, Nijmegen
    • UKGTN
    Phenotypes
    • Oculopharyngeal muscular dystrophy, OMIM:164300
    Tags
    • nucleotide-repeat-expansion
    Red PABPN1 in Arthrogryposis


    Level 2: Neurology
    Version 9.31
    Latest signed off version: v9.0 (30 Apr 2025)

    review BOTH monoallelic and biallelic, autosomal or pseudoautosomal
    Sources
    • Expert Review Red
    • UKGTN
    • Radboud University Medical Center, Nijmegen
    • Emory Genetics Laboratory
    Phenotypes
    • Oculopharyngeal muscular dystrophy, 164300
    • Oculopharyngeal muscular dystrophy
    Amber PABPN1 in Limb girdle muscular dystrophies, myofibrillar myopathies and distal myopathies


    Level 2: Neurology
    Version 5.39
    Latest signed off version: v5.0 (30 Apr 2025)

    Component of the following Super Panels:

  • Hypotonic infant
  • Other rare neuromuscular disorders
  • review MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted
    Sources
    • Expert Review Amber
    • Literature
    Phenotypes
    • Oculopharyngeal muscular dystrophy, OMIM:164300
    • oculopharyngeal muscular dystrophy, MONDO:0008116
    Tags
    • Q1_26_promote_green
    Amber PABPN1_GCN STR in Limb girdle muscular dystrophies, myofibrillar myopathies and distal myopathies


    Level 2: Neurology
    Version 5.39
    Latest signed off version: v5.0 (30 Apr 2025)

    Component of the following Super Panels:

  • Hypotonic infant
  • Other rare neuromuscular disorders
  • review BOTH monoallelic and biallelic (but BIALLELIC mutations cause a more SEVERE disease form), autosomal or pseudoautosomal
    Sources
    • Expert Review Amber
    • Literature
    Phenotypes
    • Oculopharyngeal muscular dystrophy, OMIM:164300
    • oculopharyngeal muscular dystrophy, MONDO:0008116