Protein: Glutamyl-tRNA(Gln) amidotransferase subunit A, mitochondrial (Glu-AdT subunit A) (EC 6.3.5.7) (Glutaminyl-tRNA synthase-like protein 1) (Q9H0R6)
Organism: Homo sapiens (Human)
Function: Allows the formation of correctly charged Gln-tRNA(Gln) through the transamidation of misacylated Glu-tRNA(Gln) in the mitochondria. The reaction takes place in the presence of glutamine and ATP through an activated gamma-phospho-Glu-tRNA(Gln).
Subcellular location: Mitochondrion {ECO:0000255|HAMAP-Rule:MF_03150, ECO:0000269|PubMed:19805282}.
Sequence: MLGRSLREVSAALKQGQITPTELCQKCLSLIKKTKFLNAYITVSEEVALKQAEESEKRYKNGQSLGDLDGIPIAVKDNFSTSGIETTCASNMLKGYIPPYNATVVQKLLDQGALLMGKTNLDEFAMGSGSTDGVFGPVKNPWSYSKQYREKRKQNPHSENEDSDWLITGGSSGGSAAAVSAFTCYAALGSDTGGSTRNPAAHCGLVGFKPSYGLVSRHGLIPLVNSMDVPGILTRCVDDAAIVLGALAGPDPRDSTTVHEPINKPFMLPSLADVSKLCIGIPKEYLVPELSSEVQSLWSKAADLFESEGAKVIEVSLPHTSYSIVCYHVLCTSEVASNMARFDGLQYGHRCDIDVSTEAMYAATRREGFNDVVRGRILSGNFFLLKENYENYFVKAQKVRRLIANDFVNAFNSGVDVLLTPTTLSEAVPYLEFIKEDNRTRSAQDDIFTQAVNMAGLPAVSIPVALSNQGLPIGLQFIGRAFCDQQLLTVAKWFEKQVQFPVIQLQELMDDCSAVLENEKLASVSLKQ
InterPro domains: - IPR004412: Glutamyl-tRNA(Gln) amidotransferase A subunit (family) [9-499]
- IPR036928: Amidase signature (AS) superfamily (homologous_superfamily) [3-502]
- IPR023631: Amidase signature domain (domain) [25-488]
- IPR000120: Amidase (family) [5-502]
Interaction partners: - Glutamyl-tRNA
- Glutamyl-tRNA
- Asparaginyl-tRNA synthetase
- Glutamine--tRNA ligase
- Asparagine--tRNA ligase, cytoplasmic
- Bifunctional glutamate/proline--tRNA ligase
- Aspartate--tRNA ligase, cytoplasmic
- Aspartate--tRNA ligase, mitochondrial
- Nondiscriminating glutamyl-tRNA synthetase EARS2, mitochondrial
- LIM domain-binding protein 1
Gene Ontology annotations:
Molecular Function (MF): GO:0003674 (molecular function), GO:0005488 (binding), GO:0003824 (catalytic activity), GO:0016874 (ligase activity), GO:0140640 (catalytic activity, acting on a nucleic acid), GO:0005515 (protein binding), GO:0140098 (catalytic activity, acting on RNA), GO:0016879 (ligase activity, forming carbon-nitrogen bonds), GO:0016884 (carbon-nitrogen ligase activity, with glutamine as amido-N-donor), GO:0140101 (catalytic activity, acting on a tRNA)
Biological Process (BP): GO:0008150 (biological process), GO:0008152 (metabolic process), GO:0009987 (cellular process), GO:0065007 (biological regulation), GO:0065008 (regulation of biological quality), GO:0009058 (biosynthetic process), GO:0044238 (primary metabolic process), GO:0044237 (cellular metabolic process), GO:0071704 (organic substance metabolic process), GO:0006807 (nitrogen compound metabolic process), GO:0044249 (cellular biosynthetic process), GO:0034641 (cellular nitrogen compound metabolic process), GO:0043603 (amide metabolic process), GO:1901564 (organonitrogen compound metabolic process), GO:1901576 (organic substance biosynthetic process), GO:0031647 (regulation of protein stability), GO:0044260 (cellular macromolecule metabolic process), GO:0043170 (macromolecule metabolic process), GO:0019538 (protein metabolic process), GO:1901566 (organonitrogen compound biosynthetic process), GO:0006412 (translation), GO:0009059 (macromolecule biosynthetic process), GO:0043604 (amide biosynthetic process), GO:0044271 (cellular nitrogen compound biosynthetic process), GO:0034645 (cellular macromolecule biosynthetic process), GO:0006518 (peptide metabolic process), GO:0010467 (gene expression), GO:0140053 (mitochondrial gene expression), GO:0043043 (peptide biosynthetic process), GO:0032543 (mitochondrial translation)
Cellular Component (CC): GO:0005575 (cellular component), GO:0110165 (cellular anatomical entity), GO:0005737 (cytoplasm), GO:0005829 (cytosol), GO:0005622 (intracellular anatomical structure)