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Phenylalanine--tRNA ligase beta subunit (FARSB)
- Target
- FARSB
- Molecular classification
- Enzyme, Aminoacyl-tRNA synthetase, Transferase
01
Overview
Phenylalanine--tRNA ligase beta subunit (FARSB) is a regulatory subunit of the heterotetrameric cytoplasmic enzyme phenylalanyl-tRNA synthetase, which catalyzes the attachment of L-phenylalanine to its cognate tRNA (tRNA^Phe) during protein synthesis. FARSB forms a tetrameric structure with two catalytic alpha subunits (FARSA) and two regulatory beta subunits (FARSB). The FARSB subunit is primarily involved in editing and fidelity control, preventing misincorporation of amino acids. FARSB belongs to the aminoacyl-tRNA synthetase class IIc subfamily. Pathogenic variants in FARSB cause rare recessive disorders with multi-organ symptoms, notably interstitial lung disease with brain calcifications and developmental delay. The protein is ubiquitously expressed in the cytoplasm and is essential for viability[1][2][3][4].
- Other names
- Phenylalanyl-tRNA synthetase beta subunitFARSLBFRSBPheRSPheHBPhenylalanyl-tRNA synthetase-like beta subunitPhenylalanine tRNA ligase 1 beta cytoplasmicEC 6.1.1.20HSPC173RILDBCNEDBLLA
02
Mechanism of action
Aminoacylation of tRNA (attaches L-phenylalanine to tRNA^Phe), Post-transfer editing (corrects mischarged tRNA)
03
Biological functions
Protein biosynthesistRNA aminoacylationEditing of aminoacyl-tRNAMagnesium ion binding
04
Disease associations
Interstitial lung disease with brain calcificationsLiver cirrhosisOther (rare genetic diseases)
05
Safety considerations
Essential for protein synthesis—loss of function leads to severe cellular dysfunction and is implicated in rare recessive disorders involving interstitial lung disease and neurologic findings
06
Interacting drugs
phenylalanine (as substrate)
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