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Reviewed May 2014
What is the official name of the MT-TH gene?
The official name of this gene is “mitochondrially encoded tRNA histidine.”
MT-TH is the gene's official symbol. The MT-TH gene is also known by other names, listed below.
Read more about gene names and symbols on the About page.
What is the normal function of the MT-TH gene?
The MT-TH gene provides instructions for making a particular type of RNA, a molecule that is a chemical cousin of DNA. This type of RNA, called transfer RNA (tRNA), helps assemble protein building blocks known as amino acids into full-length, functioning proteins. The MT-TH gene provides instructions for a specific form of tRNA that is designated as tRNAHis. During protein assembly, this molecule attaches to a particular amino acid, histidine (His), and inserts it into the appropriate locations in the growing protein.
The tRNAHis molecule is present in cellular structures called mitochondria. These structures convert energy from food into a form that cells can use. Through a process called oxidative phosphorylation, mitochondria use oxygen, simple sugars, and fatty acids to create adenosine triphosphate (ATP), the cell's main energy source. The tRNAHis molecule is involved in the assembly of proteins that carry out oxidative phosphorylation.
Does the MT-TH gene share characteristics with other genes?
The MT-TH gene belongs to a family of genes called TRNA (transfer RNAs).
A gene family is a group of genes that share important characteristics. Classifying individual genes into families helps researchers describe how genes are related to each other. For more information, see What are gene families? in the Handbook.
How are changes in the MT-TH gene related to health conditions?
Where is the MT-TH gene located?
The MT-TH gene is located in mitochondrial DNA.
Molecular Location in mitochondrial DNA: base pairs 12,138 to 12,206
(Homo sapiens Annotation Release 107, GRCh38.p2) (
Where can I find additional information about MT-TH?
You and your healthcare professional may find the following resources about MT-TH helpful.
You may also be interested in these resources, which are designed for genetics professionals and researchers.
What other names do people use for the MT-TH gene or gene products?
See How are genetic conditions and genes named? in the Handbook.
Where can I find general information about genes?
The Handbook provides basic information about genetics in clear language.
These links provide additional genetics resources that may be useful.
What glossary definitions help with understanding MT-TH?
acidosis ; acids ; adenine ; adenosine triphosphate ; amino acid ; ataxia ; ATP ; cardiomyopathy ; cell ; DNA ; epilepsy ; fatty acids ; gene ; guanine ; His ; histidine ; lactic acidosis ; mitochondria ; molecule ; muscle cells ; mutation ; myoclonus ; nervous system ; neurological ; nucleotide ; oxidative phosphorylation ; oxygen ; phosphorylation ; protein ; RNA ; syndrome ; transfer RNA ; tRNA
You may find definitions for these and many other terms in the Genetics Home Reference Glossary.
See also Understanding Medical Terminology.
References (9 links)
The resources on this site should not be used as a substitute for professional medical care or advice. Users seeking information about a personal genetic disease, syndrome, or condition should consult with a qualified healthcare professional. See How can I find a genetics professional in my area? in the Handbook.