MT-TG

Chr MT

tRNA-Gly

Also known as: MTTG

NCBI Gene: 4563 ENSG00000210164 OMIM: 590035

This gene encodes mitochondrial tRNA for glycine, which is essential for mitochondrial protein synthesis and oxidative phosphorylation. Mutations cause encephalomyopathy and cardiomyopathy, including hypertrophic cardiomyopathy. Inheritance is maternal through mitochondrial DNA, and disease severity depends on the degree of heteroplasmy.

OMIM ResearchSummary from Curated mito context

OMIM ResearchGenerating clinical summary…

Clinical highlights

Gene–disease validity (ClinGen)

Moderate · mitochondrial disease · MT · ClinGen 2025

Active trials

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Pubs (1 yr)

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P/LP submissions

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P/LP missense

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LOEUF

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Mechanism

Nuclear-gene analyses omitted

MT-TG is a mitochondrial tRNA gene. gnomAD constraint, carrier burden and exome coverage, the ClinVar type breakdown, the protein lollipop, AlphaFold and transcript-architecture views are built for nuclear genes and are not shown. Population data for mtDNA variants (gnomAD v3.1, homoplasmy and heteroplasmy) is on each variant page.

Where it sits in the mitochondrial genome

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proteinrRNAtRNAouter ring H-strand · inner L-strandMITOMAP confirmedreported (as of 2026-10-07)

37 genes from Ensembl (rCRS numbering, matched to MITOMAP’s Gene Loci); landmarks and disease alleles from MITOMAP (CC BY 3.0). Circular layout after MitoWheel.

ClinVar Variant Classifications

0 submitted variants in ClinVar

ClinVar

MITOMAP Disease Variants — MT-TG

Live table ↗

Variant	Disease	Status	GenBank seqs
m.9997T>C	MHCM	Uncertain significance · reported	1
m.9997T>A	Unspecified patient from clinical lab	Uncertain significance · reported	0
m.9998T>C	Suspected mitochondrial disease	Reported	0
m.10000G>A	Multisystem mitochondrial disorder	Reported	2
m.10003T>C	Hypertension / maternally inherited diabetes / hearing loss	Reported	12
m.10005A>G	Hearing loss patient	Reported	26
m.10006A>G	CIPO / Encephalopathy	Unclear	18
m.10010T>C	PEM	Uncertain significance · confirmed	0
m.10014G>A	Myopathy	Unclear	0
m.10019C>T	Hearing loss patient	Reported	0
m.10044A>G	SIDS	Unclear	194
m.10055A>G	Tic disorder patient / hearing loss patient	Reported	12
m.10057T>C	Hearing loss patient	Reported	6