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Mitochondrially encoded tRNA glutamic acid (MT-TE)

Target
MT-TE
Molecular classification
Transfer RNA (tRNA), Non-coding RNA, Other
01

Overview

Mitochondrially encoded tRNA glutamic acid (MT-TE, also called trnE or tRNAGlu) is a transfer RNA molecule encoded within human mitochondrial DNA. It consists of 68–69 nucleotides and functions by transferring the amino acid glutamic acid during mitochondrial protein synthesis, which is crucial for the assembly of proteins involved in oxidative phosphorylation, the process that produces cellular ATP. MT-TE is expressed exclusively in the mitochondria and essential for normal mitochondrial function and cellular energy homeostasis. Pathogenic mutations in MT-TE are associated with mitochondrial disorders including maternally inherited diabetes and deafness, mitochondrial myopathies, MELAS, and sometimes Leber's hereditary optic neuropathy. The molecule itself is not considered a therapeutic target (such as a receptor or enzyme), but its genetic integrity is critical for normal energy metabolism, and its mutations serve as diagnostic biomarkers for certain mitochondrial diseases.

Other names
trnEtRNAGlumt-tRNA-Glu
02

Mechanism of action

Not applicable; transfer RNAs are not direct drug targets in clinical use. Pathology arises from mutations affecting protein translation and subsequent mitochondrial dysfunction

03

Biological functions

Mitochondrial protein synthesis (translation)Oxidative phosphorylation protein assemblyEnergy metabolism regulation
04

Disease associations

Maternally inherited diabetes and deafness (MIDD)Mitochondrial myopathyLeber's hereditary optic neuropathy (LHON)Mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS)Other mitochondrial syndromes
05

Safety considerations

Mutations lead to impaired mitochondrial function, manifesting as energy deficits, myopathy, neurological symptoms, diabetes, and deafnessNo known therapeutic interventions currently target this RNA directly, so safety concerns pertain mainly to the risk of disease due to inherited mutations and secondary drug sensitivities (e.g., caution with drugs impacting mitochondrial function in susceptible patients)
06

Biomarkers

Pathogenic mutations (e.g., m.14709T>C, m.14739G>A) can be used as genetic biomarkers for mitochondrial disease, including maternally inherited diabetes and deafness, myopathy, and other syndromes

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