UBA1

ubiquitin like modifier activating enzyme 1
OMIM: 314370, Gene2Phenotype

6 panels

Panel Reviews Mode of inheritance Details
6 panels
Red UBA1 in Primary immunodeficiency or monogenic inflammatory bowel disease


Level 2: Immunology
Version 8.78
Latest signed off version: v8.0 (30 Apr 2025)

review Other
Sources
  • Expert Review Red
  • Literature
Phenotypes
  • VEXAS syndrome, somatic, OMIM:301054
Tags
  • somatic
Green UBA1 in Arthrogryposis


Level 2: Neurology
Version 9.24
Latest signed off version: v9.0 (30 Apr 2025)

review X-LINKED: hemizygous mutation in males, biallelic mutations in females
Sources
  • Expert Review Green
  • Radboud University Medical Center, Nijmegen
  • Illumina TruGenome Clinical Sequencing Services
  • UKGTN
  • Expert list
Phenotypes
  • Spinal muscular atrophy, X-linked 2, infantile 301830
Green UBA1 in Fetal anomalies


Level 2: Fetal (including NIPD)
Version 6.140
Latest signed off version: v6.0 (30 Apr 2025)

review X-LINKED: hemizygous mutation in males, biallelic mutations in females
Sources
  • PAGE Additional Gene List
  • Expert Review Green
Phenotypes
  • Spinal muscular atrophy, X-linked 2, infantile 301830
Green UBA1 in Paediatric motor neuronopathies


Level 2: Neurology
Version 3.12
Latest signed off version: v3.9 (30 Apr 2025)

Component of the following Super Panels:

  • Hypotonic infant
  • Other rare neuromuscular disorders
  • review X-LINKED: hemizygous mutation in males, monoallelic mutations in females may cause disease (may be less severe, later onset than males)
    Sources
    • Expert Review Green
    • Radboud University Medical Center, Nijmegen
    • Illumina TruGenome Clinical Sequencing Services
    Phenotypes
    • Spinal muscular atrophy, X-linked 2, infantile, OMIM:301830
    Green UBA1 in Hereditary neuropathy or pain disorder


    Level 2: Neurology
    Version 7.36
    Latest signed off version: v7.0 (30 Apr 2025)

    review X-LINKED: hemizygous mutation in males, biallelic mutations in females
    Sources
    • NHS GMS
    • Expert Review Green
    • Expert list
    Phenotypes
    • Spinal muscular atrophy, X-linked 2, infantile, OMIM:301830
    Green UBA1 in Autoinflammatory disorders


    Level 2: Immunology
    Version 2.35
    Latest signed off version: v2.0 (1 May 2024)

    review Other
    Sources
    • Expert Review Green
    • NHS GMS
    Phenotypes
    • VEXAS syndrome, somatic, OMIM:301054
    Tags
    • somatic