OPLAH

5-oxoprolinase, ATP-hydrolysing
OMIM: 614243, Gene2Phenotype

3 panels

Panel Reviews Mode of inheritance Details
3 panels
Amber OPLAH in Undiagnosed metabolic disorders

Level 3: Specific metabolic abnormalities
Level 2: Metabolic disorders
Version 1.643

review BOTH monoallelic and biallelic, autosomal or pseudoautosomal
Sources
  • Expert Review Amber
  • Radboud University Medical Center, Nijmegen
  • Literature
Phenotypes
  • 5-oxoprolinase deficiency 260005
Amber OPLAH in Likely inborn error of metabolism


Level 2: Metabolic
Version 8.103
Latest signed off version: v8.0 (30 Apr 2025)

Component of the following Super Panels:

  • Childhood onset leukodystrophy
  • Hypotonic infant
  • Paediatric disorders
  • Unexplained death in infancy and sudden unexplained death in childhood
  • review BOTH monoallelic and biallelic, autosomal or pseudoautosomal
    Sources
    • Expert Review Amber
    • London North GLH
    • NHS GMS
    Phenotypes
    • 5-oxoprolinase deficiency 260005
    Red OPLAH in Childhood onset dystonia, chorea or related movement disorder


    Level 2: Neurology
    Version 7.18
    Latest signed off version: v7.0 (30 Apr 2025)

    review Not set
    Sources
    • Expert Review Red
    • London North GLH