1. Genes and Genomic Entities
  2. MT-TV

MT-TV

mitochondrially encoded tRNA valine
OMIM: 590105, Gene2Phenotype

7 panels

Panel Reviews Mode of inheritance Details
7 panels
No list MT-TV in Hypertrophic cardiomyopathy


Level 2: Cardiology
Version 6.2
Latest signed off version: v6.0 (6 May 2026)

Component of the following Super Panels:

  • Sudden unexplained death or survivors of a cardiac event
  • Unexplained death in infancy and sudden unexplained death in childhood

    		•  review MITOCHONDRIAL
    Sources
    • Expert Review Removed
    • Literature
    Phenotypes
    • MELAS syndrome, MONDO:0010789
    • hypertrophic cardiomyopathy, MONDO:000504
    Tags
    • locus-type-rna-transfer
    • curated_removed
    Green MT-TV in Undiagnosed metabolic disorders

    Level 3: Specific metabolic abnormalities
    Level 2: Metabolic disorders
    Version 1.645

    		review MITOCHONDRIAL
    Sources
    • Expert Review Green
    Tags
    • locus-type-rna-transfer
    Green MT-TV in Likely inborn error of metabolism


    Level 2: Metabolic
    Version 9.4
    Latest signed off version: v9.0 (6 May 2026)

    Component of the following Super Panels:

  • Childhood onset leukodystrophy
  • Hypotonic infant
  • Paediatric disorders
  • Unexplained death in infancy and sudden unexplained death in childhood

    		•  review MITOCHONDRIAL
    Sources
    • Expert Review Green
    Tags
    • locus-type-rna-transfer
    • gene-checked
    Green MT-TV in Mitochondrial disorders


    Level 2: Mitochondrial
    Version 10.4
    Latest signed off version: v10.0 (6 May 2026)

    Component of the following Super Panels:

  • Childhood onset leukodystrophy

    		•  review MITOCHONDRIAL
    Sources
    • Expert Review Green
    • UKGTN
    Tags
    • locus-type-rna-transfer
    • gene-checked
    Amber MT-TV in Paediatric or syndromic cardiomyopathy


    Level 2: Cardiology
    Version 8.1
    Latest signed off version: v8.0 (6 May 2026)

    Component of the following Super Panels:

  • Unexplained death in infancy and sudden unexplained death in childhood

    		•  review MITOCHONDRIAL
    Sources
    • Expert Review Amber
    • Literature
    Phenotypes
    • MELAS syndrome, MONDO:0010789
    • hypertrophic cardiomyopathy, MONDO:000504
    Tags
    • locus-type-rna-transfer
    No list MT-TV in Hereditary neuropathy or pain disorder


    Level 2: Neurology
    Version 8.1
    Latest signed off version: v8.0 (6 May 2026)

    		review MITOCHONDRIAL
    Sources
    • Other
    Phenotypes
    • peripheral neuropathy
    • spasticity
    No list MT-TV in Childhood onset dystonia, chorea or related movement disorder


    Level 2: Neurology
    Version 8.3
    Latest signed off version: v8.0 (6 May 2026)

    		review MITOCHONDRIAL
    Sources
    • Expert Review Removed
    • London North GLH
    Tags
    • curated_removed