FTSJ1
Description
The FTSJ1 (FtsJ RNA 2'-O-methyltransferase 1) is a protein-coding gene located on chromosome X.
Putative ribosomal RNA methyltransferase 1 is an enzyme that in humans is encoded by the FTSJ1 gene. The protein encoded by this gene is a member of the S-adenosylmethionine-binding protein family. It is a nucleolar protein and may be involved in the processing and modification of rRNA. Three alternatively spliced transcript variants encoding different isoforms have been described for this gene.
Methylates the 2'-O-ribose of nucleotides at positions 32 and 34 of the tRNA anticodon loop of substrate tRNAs. Essential for accurate protein synthesis in the cytoplasm. Requires THADA for methylation at position 32 and WDR6 for methylation at position 34. Enhances translation of the UUU codon. Plays a role in neurogenesis. Required for the expression of genes involved in neurogenesis, mitochondrial translation and energy production, and lipid biosynthesis. Necessary for RNA-mediated gene silencing. May modify position 32 in tRNA(Arg(ACG)), tRNA(Arg(CCG)), tRNA(Arg(UCG)), tRNA(Cys(GCA)), tRNA(Cys(ACA)), tRNA(Gln(CUG)), tRNA(Gln(UUG)), tRNA(Gly(CCC)), tRNA(Leu(CAG))/tRNA(Leu(CAA)), tRNA(Leu(A/IAG)), tRNA(Leu(UAG)), tRNA(Phe(GAA)), tRNA(Pro(AGG))/tRNA(Pro(CGG))/tRNA(Pro(UGG)) and tRNA(Trp(CCA)), and position 34 in tRNA(Phe(GAA)), tRNA(Leu(CAA)), tRNA(Sec(UCA)), and tRNA(Trp(CCA)). Interacts directly with WDR6, which is required for 2'-O-methylation at position 34 in substrate tRNAs.
FTSJ1 is also known as CDLIV, JM23, MRX44, MRX9, SPB1, TRMT7, XLID9.
