Recombinant Human Serine–tRNA ligase, mitochondrial (SARS2)

Uniprot Id

Q9NP81

Target Species

Human

Target Name

SARS2

Target Full Name

Serine--tRNA ligase, mitochondrial

Target Function

Catalyzes the attachment of serine to tRNA(Ser). Is also probably able to aminoacylate tRNA(Sec) with serine, to form the misacylated tRNA L-seryl-tRNA(Sec), which will be further converted into selenocysteinyl-tRNA(Sec).

Target Involvement

Hyperuricemia, pulmonary hypertension, renal failure, and alkalosis syndrome (HUPRAS)

Target Subcellular Location

Mitochondrion matrix.

Target Protein Families

Class-II aminoacyl-tRNA synthetase family, Type-1 seryl-tRNA synthetase subfamily

Target Synonyms

mitochondrial; mtSerRS ; SARS ; SARS2; SARSM ; Serine tRNA ligase; Serine--tRNA ligase; SerRS; SerRSmt; SERS ; Seryl tRNA synthetase 2; Seryl-tRNA synthetase; Seryl-tRNA(Ser/Sec) synthetase; SYS ; SYSM_HUMAN

Target Background

This gene encodes the mitochondrial seryl-tRNA synthethase precursor, a member of the class II tRNA synthetase family. The mature enzyme catalyzes the ligation of Serine to tRNA(Ser) and participates in the biosynthesis of selenocysteinyl-tRNA(sec) in mitochondria. The enzyme contains an N-terminal tRNA binding domain and a core catalytic domain. It functions in a homodimeric form, which is stabilized by tRNA binding. This gene is regulated by a bidirectional promoter that also controls the expression of mitochondrial ribosomal protein S12. Both genes are within the critical interval for the autosomal dominant deafness locus DFNA4 and might be linked to this disease. Multiple transcript variants encoding different isoforms have been identified for this gene.