Names & Taxonomy
- Uniprot ID:
- O95363
- Entry Name:
- SYFM_HUMAN
- Status:
- reviewed
- Protein Names:
- Phenylalanine--tRNA ligase, mitochondrial (EC 6.1.1.20) (Phenylalanyl-tRNA synthetase) (PheRS)
- Gene Names:
- FARS2 FARS1 HSPC320
- Gene Names Orf:
- HSPC320
- Gene Names Primary:
- FARS2
- Organism:
- Homo sapiens (Human)
Structure
- Length:
- 451
- Sequence:
- MVGSALRRGAHAYVYLVSKASHISRGHQHQAWGSRPPAAECATQRAPGSVVELLGKSYPQDDHSNLTRKVLTRVGRNLHNQQHHPLWLIKERVKEHFYKQYVGRFGTPLFSVYDNLSPVVTTWQNFDSLLIPADHPSRKKGDNYYLNRTHMLRAHTSAHQWDLLHAGLDAFLVVGDVYRRDQIDSQHYPIFHQLEAVRLFSKHELFAGIKDGESLQLFEQSSRSAHKQETHTMEAVKLVEFDLKQTLTRLMAHLFGDELEIRWVDCYFPFTHPSFEMEINFHGEWLEVLGCGVMEQQLVNSAGAQDRIGWAFGLGLERLAMILYDIPDIRLFWCEDERFLKQFCVSNINQKVKFQPLSKYPAVINDISFWLPSENYAENDFYDLVRTIGGDLVEKVDLIDKFVHPKTHKTSHCYRITYRHMERTLSQREVRHIHQALQEAAVQLLGVEGRF
- Proteomes:
- UP000005640
Subcellular location
- Subcellular Location:
- Mitochondrion matrix
Function
- Function:
- Is responsible for the charging of tRNA(Phe) with phenylalanine in mitochondrial translation. To a lesser extent, also catalyzes direct attachment of m-Tyr (an oxidized version of Phe) to tRNA(Phe), thereby opening the way for delivery of the misacylated tRNA to the ribosome and incorporation of ROS-damaged amino acid into proteins.
- Catalytic Activity:
- ATP + L-phenylalanine + tRNA(Phe) = AMP + diphosphate + L-phenylalanyl-tRNA(Phe).
- Kinetics:
- BIOPHYSICOCHEMICAL PROPERTIES: ; Kinetic parameters: KM=2.2 uM for L-phenylalanine
- Cross Reference Drug Bank:
- DB00120
- Gene Ontology Go:
- mitochondrial matrix
mitochondrion
ATP binding
magnesium ion binding
phenylalanine-tRNA ligase activity
tRNA binding
gene expression
phenylalanyl-tRNA aminoacylation
tRNA aminoacylation for protein translation
tRNA processing - Gene Ontology Biological Process:
- gene expression
phenylalanyl-tRNA aminoacylation
tRNA aminoacylation for protein translation
tRNA processing - Gene Ontology Molecular Function:
- ATP binding
magnesium ion binding
phenylalanine-tRNA ligase activity
tRNA binding - Gene Ontology Cellular Component:
- mitochondrial matrix
mitochondrion - Keywords:
- 3D-structure
ATP-binding
Acetylation
Aminoacyl-tRNA synthetase
Complete proteome
Disease mutation
Ligase
Mitochondrion
Nucleotide-binding
Polymorphism
Protein biosynthesis
Reference proteome
Transit peptide - Interacts With:
- Q6UY14-3; Q6RW13; Q9UKG1; Q13137; Q96DZ9; Q6NT76; Q9UKT9; A1A4E9; Q15323; Q6A162; P60370; P60409; P60411; Q9UJV3-2; Q13064; Q7Z3S9; O95199; O75558; O75478; Q12800; P14373; Q9BYV2