Harmonizome 3.0

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]

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]

phosphoribosyl pyrophosphate amidotransferase pseudogene 2

phosphoribosyl pyrophosphate amidotransferase

The protein encoded by this gene is a member of the purine/pyrimidine phosphoribosyltransferase family. It is a regulatory allosteric enzyme that catalyzes the first step of de novo purine nucleotide biosythetic pathway. This gene and PAICS/AIRC gene, a bifunctional enzyme catalyzing steps six and seven of this pathway, are located in close proximity on chromosome 4, and divergently transcribed from an intergenic region. [provided by RefSeq, Mar 2011]

phosphoribosyl pyrophosphate amidotransferase pseudogene

phosphoribosyl pyrophosphate amidotransferase pseudogene 1

glutamine amidotransferase class 1 domain containing 1

Predicted to enable glyoxalase III activity. Predicted to be involved in methylglyoxal catabolic process to D-lactate via S-lactoyl-glutathione. Located in extracellular exosome. [provided by Alliance of Genome Resources, Mar 2025]

glutamine amidotransferase class 1 domain containing 3

This gene encodes a potential mitochondrial protein that is a member of the DJ-1/PfpI gene family. This protein is overexpressed in fetal Down syndrome brain. Alternate splicing results in multiple transcript variants. [provided by RefSeq, May 2010]

glutamyl aminopeptidase (aminopeptidase A) pseudogene

gamma-glutamyl hydrolase (conjugase, folylpolygammaglutamyl hydrolase)

This gene catalyzes the hydrolysis of folylpoly-gamma-glutamates and antifolylpoly-gamma-glutamates by the removal of gamma-linked polyglutamates and glutamate. [provided by RefSeq, Jul 2008]

glutamyl aminopeptidase (aminopeptidase A) pseudogene

glutamyl aminopeptidase (aminopeptidase A)

The ENPEP gene encodes glutamyl aminopeptidase, a type II integral membrane protein with an extracellular zinc-binding domain. This protein can upregulate blood pressure by cleaving the N-terminal aspartate from angiotensin II, and can regulate blood vessel formation and enhance tumorigenesis in some tissues. Along with ANPEP and DPP4, ENPEP was found to be a candidate co-receptor for the coronavirus SARS-CoV-2, which causes COVID-19. [provided by RefSeq, Apr 2020]

gamma-glutamyl carboxylase

This gene encodes an integral membrane protein of the rough endoplasmic reticulum that carboxylates glutamate residues of vitamin K-dependent proteins to gamma carboxyl glutamate, a modification that is required for their activity. The vitamin K-dependent protein substrates have a propeptide that binds the enzyme, with carbon dioxide, dioxide, and reduced vitamin K acting as co-substrates. Vitamin K-dependent proteins affect a number of physiologic processes including blood coagulation, prevention of vascular calcification, and inflammation. Allelic variants of this gene have been associated with pseudoxanthoma elasticum-like disorder with associated multiple coagulation factor deficiency. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Aug 2015]

transfer RNA-Gln (CTG) 8-2

transfer RNA-Gln (CTG) 18-1

transfer RNA-Gln (CTG) 1-5

transfer RNA-Gln (CTG) 9-1

transfer RNA-Gln (CTG) 15-1

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

transfer RNA-Gln (CTG) 14-1

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

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

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

transfer RNA-Gln (TTG) 8-1

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

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

transfer RNA-Gln (CTG) 4-1

transfer RNA-Gln (CTG) 4-2

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

transfer RNA-Gln (CTG) 3-2

transfer RNA-Gln (CTG) 1-4

transfer RNA-Gln (CTG) 1-1

transfer RNA-Gln (CTG) 1-2

transfer RNA-Gln (CTG) 1-3

transfer RNA-Gln (TTG) 3-3

transfer RNA-Gln (TTG) 3-2

transfer RNA-Gln (TTG) 3-1

transfer RNA-Gln (TTG) 2-1

transfer RNA-Gln (CTG) 5-1

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

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

transfer RNA-Gln (CTG) 17-1

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

transfer RNA-Gln (CTG) 16-1

transfer RNA-Gln (TTG) 7-1

transfer RNA-Gln (TTG) 6-1

transfer RNA-Gln (CTG) 13-1

transfer RNA-Gln (CTG) 11-1

transfer RNA-Gln (CTG) 10-1

transfer RNA-Gln (CTG) 12-1

transfer RNA-Gln (CTG) 8-1

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

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

transfer RNA-Gln (CTG) 7-1

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]

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

transfer RNA-Gln (CTG) 2-1

transfer RNA-Gln (CTG) 3-1

transfer RNA-Gln (TTG) 5-1

transfer RNA-Gln (CTG) 6-1

transfer RNA-Gln (TTG) 9-1

transfer RNA-Gln (TTG) 4-1

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

transfer RNA-Gln (TTG) 10-1

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

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