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 9.9
Latest signed off version: v9.0 (6 May 2026)

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


Level 2: Neurology
Version 10.5
Latest signed off version: v10.0 (6 May 2026)

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 7.8
Latest signed off version: v7.0 (6 May 2026)

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 3.14
Latest signed off version: v3.13 (6 May 2026)

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 8.1
    Latest signed off version: v8.0 (6 May 2026)

    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 3.7
    Latest signed off version: v3.0 (6 May 2026)

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