Gene
MT-TP
mitochondrially encoded tRNA-Pro (CCN)
- HGNC 7494↗(opens in a new tab)
- Ensembl gene ENSG00000210196↗(opens in a new tab)
- NCBI Gene 4571↗(opens in a new tab)
Summary
- Cytogenetic location
- mitochondria
- Inheritance
- Mitochondrial
- Also known as
- trnP
- Previous symbols
- MTTP
- Locus type
- RNA, transfer
Reference genomeGRCh38hg38
Positions on gene and variant pages follow this choice, in this browser.
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Primary Mitochondrial Disease
- MERRF SyndromeMitochondrial
Usually caused by variants in mitochondrial DNA such as m.8344A>G, which can cause muscle jerks (myoclonus), seizures, balance problems and muscle weakness.
- 1 GenCC submitter rates it definitive or strong
What knowing changes
Knowing guides seizure treatment choices and monitoring of heart and hearing.
Broader groupings
Epilepsy (Full Panel)
- Epilepsy
One of 171 genes linked to it
A brain disorder characterized by episodes of abnormally increased neuronal discharge resulting in transient episodes of sensory or motor neurological dysfunction, or psychic dysfunction. These episodes may or may not be associated with loss of consciousness or convulsions.
- 1 GenCC submitter rates it definitive or strong
Inheritance
- Mitochondrial
- The change is in the mitochondria's own DNA, which children inherit from their mother alone. A mother passes it on to all of her children, in amounts that can vary; a father passes it on to none.
How a genome test reads MT-TP
1
position in MT-TP where ClinVar classes a change as pathogenic or likely pathogenic
None of them falls in a region short reads find hard.
Genes linked to the same conditions
- AARS1alanyl-tRNA synthetase 1Shares 1 condition
- ABAT4-aminobutyrate aminotransferaseShares 1 condition
- ACTL6Bactin like 6BShares 1 condition
- ADAM22ADAM metallopeptidase domain 22Shares 1 condition
- ALDH7A1aldehyde dehydrogenase 7 family member A1Shares 1 condition
- ALG13ALG13 UDP-N-acetylglucosaminyltransferase subunitShares 1 condition
- AP3B2adaptor related protein complex 3 subunit beta 2Shares 1 condition
- ARHGEF9Cdc42 guanine nucleotide exchange factor 9Shares 1 condition
- ARV1ARV1 fatty acid homeostasis modulatorShares 1 condition
- ARXaristaless related homeoboxShares 1 condition
- ATP1A2ATPase Na+/K+ transporting subunit alpha 2Shares 1 condition
- ATP1A3ATPase Na+/K+ transporting subunit alpha 3Shares 1 condition
Traits linked to MT-TP
The Human Phenotype Ontology lists these as features of the rare conditions MT-TP can cause. Each trait's page lists the other genes linked to it.
External links
Where this comes from
- HGNC: the gene's symbol, name, other symbols and location.
- GenCC and ClinGen, released 2026-10-16: which conditions the gene is linked to, and how strongly.
- The ACMG secondary findings list, SF v3.2.
- ClinVar and the GIAB stratifications: known disease-causing sites, and the regions short reads find hard.
- The Human Phenotype Ontology, release 2026-09-01: the traits linked to the gene. Find out more at hpo.jax.org.
- Data release 2026.10.16-c6eea6bf.
Cite this page
Dante Labs. (n.d.). MT-TP gene: mitochondrially encoded tRNA-Pro (CCN). Retrieved 16 October 2026, from https://dantelabs.com/genes/mt-tp
Dante Labs. MT-TP gene: mitochondrially encoded tRNA-Pro (CCN) [Internet]. [cited 16 October 2026]. Available from: https://dantelabs.com/genes/mt-tp
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