ID:SYSM_HUMAN DESCRIPTION: RecName: Full=Serine--tRNA ligase, mitochondrial; EC=6.1.1.11; AltName: Full=SerRSmt; AltName: Full=Seryl-tRNA synthetase; Short=SerRS; AltName: Full=Seryl-tRNA(Ser/Sec) synthetase; Flags: Precursor; FUNCTION: Catalyzes the attachment of serine to tRNA(Ser). Is also 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) (By similarity). CATALYTIC ACTIVITY: ATP + L-serine + tRNA(Ser) = AMP + diphosphate + L-seryl-tRNA(Ser). CATALYTIC ACTIVITY: ATP + L-serine + tRNA(Sec) = AMP + diphosphate + L-seryl-tRNA(Sec). PATHWAY: Aminoacyl-tRNA biosynthesis; selenocysteinyl-tRNA(Sec) biosynthesis; L-seryl-tRNA(Sec) from L-serine and tRNA(Sec): step 1/1. SUBUNIT: Homodimer. The tRNA molecule binds across the dimer (By similarity). INTERACTION: Q9UHX1:PUF60; NbExp=1; IntAct=EBI-1049768, EBI-1053259; SUBCELLULAR LOCATION: Mitochondrion matrix (By similarity). DOMAIN: Consists of two distinct domains, a catalytic core and a N-terminal extension that is involved in tRNA binding (By similarity). DISEASE: Defects in SARS2 are the cause of hyperuricemia pulmonary hypertension renal failure and alkalosis (HUPRA) [MIM:613845]. HUPRA is a multisystem disorder characterized by onset in infancy of progressive renal failure leading to electrolyte imbalances, metabolic alkalosis, pulmonary hypertension, hypotonia, and delayed development. Affected individuals are born prematurely. SIMILARITY: Belongs to the class-II aminoacyl-tRNA synthetase family. Type-1 seryl-tRNA synthetase subfamily. SEQUENCE CAUTION: Sequence=AAH01020.2; Type=Erroneous initiation; Note=Translation N-terminally extended;
The RNAfold program from the Vienna RNA Package is used to perform the secondary structure predictions and folding calculations. The estimated folding energy is in kcal/mol. The more negative the energy, the more secondary structure the RNA is likely to have.
Pfam Domains: PF00587 - tRNA synthetase class II core domain (G, H, P, S and T)
SCOP Domains: 46589 - tRNA-binding arm 55681 - Class II aaRS and biotin synthetases
ModBase Predicted Comparative 3D Structure on Q9NP81
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Orthologous Genes in Other Species
Orthologies between human, mouse, and rat are computed by taking the best BLASTP hit, and filtering out non-syntenic hits. For more distant species reciprocal-best BLASTP hits are used. Note that the absence of an ortholog in the table below may reflect incomplete annotations in the other species rather than a true absence of the orthologous gene.
US Server
Sequence and Links to Tools and Databases 
Common Gene Haplotype Alleles 
