FARS2 Rabbit Polyclonal Antibody (HA500261)

UNIPROT #

O95363

PROTEIN NAME

FARS2

SYNONYMS

dJ236A3.1 (phenylalanine tRNA synthetase) antibody;dJ520B18.2 (FARS1 (phenylalanine tRNA synthetase)) antibody;FARS1 antibody;Fars2 antibody;HSPC320 antibody;Phenylalanine translase antibody;Phenylalanine tRNA ligase 2, mitochondrial antibody;Phenylalanine tRNA ligase antibody;Phenylalanine tRNA synthetase 1 (mitochondrial) antibody;Phenylalanine tRNA synthetase 2 (mitochondrial) antibody;Phenylalanine--tRNA ligase antibody;Phenylalanyl tRNA synthetase 2 antibody;Phenylalanyl-tRNA synthetase, mitochondrial antibody;PheRS antibody;SYFM_HUMAN antibody

SUBCELLULAR LOCATION

Mitochondrion matrix, Mitochondrion.

FUNCTION

This gene encodes a protein that transfers phenylalanine to its cognate tRNA. This protein localizes to the mitochondrion and plays a role in mitochondrial protein translation. Mutations in this gene can cause combined oxidative phosphorylation deficiency 14 (Alpers encephalopathy). Alternative splicing results in multiple transcript variants. 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.