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Congenital muscular dystrophy and congenital myopathy

Gene: PABPN1

Red List (low evidence)

PABPN1 (poly(A) binding protein nuclear 1)
EnsemblGeneIds (GRCh38): ENSG00000100836
EnsemblGeneIds (GRCh37): ENSG00000100836
OMIM: 602279, Gene2Phenotype
PABPN1 is in 3 panels

2 reviews

Ellen McDonagh (Genomics England Curator)

Comment on list classification: Demoted from green to red due to expert review.
Created: 19 Dec 2016, 12:42 p.m.

Emma Clement (Great Ormond Street Hospital)

Red List (low evidence)

not a CMD presentation
Created: 19 Dec 2016, 11:47 a.m.

Mode of inheritance
BOTH monoallelic and biallelic, autosomal or pseudoautosomal

Phenotypes
Oculopharyngeal muscular dystrophy, 164300; Oculopharyngeal muscular dystrophy

Details

Mode of Inheritance
BOTH monoallelic and biallelic, autosomal or pseudoautosomal
Sources
  • Radboud University Medical Center, Nijmegen
  • Expert Review Red
  • Emory Genetics Laboratory
  • UKGTN
Phenotypes
  • Oculopharyngeal muscular dystrophy, OMIM:164300
Tags
nucleotide-repeat-expansion
OMIM
602279
Clinvar variants
Variants in PABPN1
Penetrance
Complete
Panels with this gene

History Filter Activity

6 Feb 2023, Gel status: 1

Created, Added New Source, Added Tag, Set mode of inheritance, Set Phenotypes, Set penetrance

Arina Puzriakova (Genomics England Curator)

gene: PABPN1 was added gene: PABPN1 was added to Congenital muscular dystrophy and congenital myopathy. Sources: UKGTN,Emory Genetics Laboratory,Expert Review Red,Radboud University Medical Center, Nijmegen nucleotide-repeat-expansion tags were added to gene: PABPN1. Mode of inheritance for gene: PABPN1 was set to BOTH monoallelic and biallelic, autosomal or pseudoautosomal Phenotypes for gene: PABPN1 were set to Oculopharyngeal muscular dystrophy, OMIM:164300 Penetrance for gene: PABPN1 were set to Complete