Name

MTHFS Gene

5,10-methenyltetrahydrofolate synthetase (5-formyltetrahydrofolate cyclo-ligase)

The protein encoded by this gene is an enzyme that catalyzes the conversion of 5-formyltetrahydrofolate to 5,10-methenyltetrahydrofolate, a precursor of reduced folates involved in 1-carbon metabolism. An increased activity of the encoded protein can result in an increased folate turnover rate and folate depletion. Three transcript variants encoding two different isoforms have been found for this gene. [provided by RefSeq, Jun 2011]

TRQ-CTG8-2 Gene

transfer RNA-Gln (CTG) 8-2

TRQ-CTG18-1 Gene

transfer RNA-Gln (CTG) 18-1

TRQ-CTG1-5 Gene

transfer RNA-Gln (CTG) 1-5

TRQ-CTG9-1 Gene

transfer RNA-Gln (CTG) 9-1

TRQ-CTG15-1 Gene

transfer RNA-Gln (CTG) 15-1

NMTRQ-TTG8-1 Gene

nuclear-encoded mitochondrial transfer RNA-Gln (TTG) 8-1

TRQ-CTG14-1 Gene

transfer RNA-Gln (CTG) 14-1

NMTRQ-TTG2-1 Gene

nuclear-encoded mitochondrial transfer RNA-Gln (TTG) 2-1

NMTRQ-TTG3-1 Gene

nuclear-encoded mitochondrial transfer RNA-Gln (TTG) 3-1

NMTRQ-TTG12-1 Gene

nuclear-encoded mitochondrial transfer RNA-Gln (TTG) 12-1

TRQ-TTG8-1 Gene

transfer RNA-Gln (TTG) 8-1

NMTRQ-TTG6-1 Gene

nuclear-encoded mitochondrial transfer RNA-Gln (TTG) 6-1

NMTRQ-TTG13-1 Gene

nuclear-encoded mitochondrial transfer RNA-Gln (TTG) 13-1

TRQ-CTG4-1 Gene

transfer RNA-Gln (CTG) 4-1

TRQ-CTG4-2 Gene

transfer RNA-Gln (CTG) 4-2

NMTRQ-TTG7-1 Gene

nuclear-encoded mitochondrial transfer RNA-Gln (TTG) 7-1

TRQ-CTG3-2 Gene

transfer RNA-Gln (CTG) 3-2

TRQ-CTG1-4 Gene

transfer RNA-Gln (CTG) 1-4

TRQ-CTG1-1 Gene

transfer RNA-Gln (CTG) 1-1

TRQ-CTG1-2 Gene

transfer RNA-Gln (CTG) 1-2

TRQ-CTG1-3 Gene

transfer RNA-Gln (CTG) 1-3

TRQ-TTG3-3 Gene

transfer RNA-Gln (TTG) 3-3

TRQ-TTG3-2 Gene

transfer RNA-Gln (TTG) 3-2

TRQ-TTG3-1 Gene

transfer RNA-Gln (TTG) 3-1

TRQ-TTG2-1 Gene

transfer RNA-Gln (TTG) 2-1

TRQ-CTG5-1 Gene

transfer RNA-Gln (CTG) 5-1

GATB Gene

glutamyl-tRNA(Gln) amidotransferase, subunit B

Contributes to glutaminyl-tRNA synthase (glutamine-hydrolyzing) activity. Involved in glutaminyl-tRNAGln biosynthesis via transamidation and mitochondrial translation. Located in mitochondrion. Part of glutamyl-tRNA(Gln) amidotransferase complex. Implicated in combined oxidative phosphorylation deficiency 41. [provided by Alliance of Genome Resources, Mar 2025]

GATC Gene

glutamyl-tRNA(Gln) amidotransferase, subunit C

Predicted to enable ATP binding activity and glutaminyl-tRNA synthase (glutamine-hydrolyzing) activity. Involved in glutaminyl-tRNAGln biosynthesis via transamidation and mitochondrial translation. Located in mitochondrion. Part of glutamyl-tRNA(Gln) amidotransferase complex. Implicated in combined oxidative phosphorylation deficiency 42. [provided by Alliance of Genome Resources, Mar 2025]

NMTRQ-TTG11-1 Gene

nuclear-encoded mitochondrial transfer RNA-Gln (TTG) 11-1

NMTRQ-TTG10-1 Gene

nuclear-encoded mitochondrial transfer RNA-Gln (TTG) 10-1

TRQ-CTG17-1 Gene

transfer RNA-Gln (CTG) 17-1

NMTRQ-TTG9-1 Gene

nuclear-encoded mitochondrial transfer RNA-Gln (TTG) 9-1

TRQ-CTG16-1 Gene

transfer RNA-Gln (CTG) 16-1

TRQ-TTG7-1 Gene

transfer RNA-Gln (TTG) 7-1

TRQ-TTG6-1 Gene

transfer RNA-Gln (TTG) 6-1

TRQ-CTG13-1 Gene

transfer RNA-Gln (CTG) 13-1

TRQ-CTG11-1 Gene

transfer RNA-Gln (CTG) 11-1

TRQ-CTG10-1 Gene

transfer RNA-Gln (CTG) 10-1

TRQ-CTG12-1 Gene

transfer RNA-Gln (CTG) 12-1

TRQ-CTG8-1 Gene

transfer RNA-Gln (CTG) 8-1

NMTRQ-TTG1-1 Gene

nuclear-encoded mitochondrial transfer RNA-Gln (TTG) 1-1

NMTRQ-TTG15-1 Gene

nuclear-encoded mitochondrial transfer RNA-Gln (TTG) 15-1

TRQ-CTG7-1 Gene

transfer RNA-Gln (CTG) 7-1

TRQ-TTG1-1 Gene

transfer RNA-Gln (TTG) 1-1

Mammalian cells contain at least 2 species of glutamine tRNA, the Gln(CUG) form (TRQ1; MIM 189919) and the Gln(UUG) form. Nemoto et al. (1991) [PubMed 2041751] reported the nucleotide sequence of the human tRNA(Gln/UUG) gene. In a correction, Nemoto et al. (1991) [PubMed 2041751] stated that the gene involved should have been designated tRNA-GLN/U*UG, where U* is a derivative of U.[supplied by OMIM, Sep 2009]

NMTRQ-TTG14-1 Gene

nuclear-encoded mitochondrial transfer RNA-Gln (TTG) 14-1

TRQ-CTG2-1 Gene

transfer RNA-Gln (CTG) 2-1

TRQ-CTG3-1 Gene

transfer RNA-Gln (CTG) 3-1

TRQ-TTG5-1 Gene

transfer RNA-Gln (TTG) 5-1

TRQ-CTG6-1 Gene

transfer RNA-Gln (CTG) 6-1

TRQ-TTG9-1 Gene

transfer RNA-Gln (TTG) 9-1

TRQ-TTG4-1 Gene

transfer RNA-Gln (TTG) 4-1

NMTRQ-CTG1-1 Gene

nuclear-encoded mitochondrial transfer RNA-Gln (CTG) 1-1

TRQ-TTG10-1 Gene

transfer RNA-Gln (TTG) 10-1

NMTRQ-TTG4-1 Gene

nuclear-encoded mitochondrial transfer RNA-Gln (TTG) 4-1

NMTRQ-TTG5-1 Gene

nuclear-encoded mitochondrial transfer RNA-Gln (TTG) 5-1