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Protein

Serine--tRNA ligase, mitochondrial

Gene

SARS2

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

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).By similarity

Caution

Catalytic activityi

ATP + L-serine + tRNA(Ser) = AMP + diphosphate + L-seryl-tRNA(Ser).By similarity
ATP + L-serine + tRNA(Sec) = AMP + diphosphate + L-seryl-tRNA(Sec).By similarity

Pathwayi: selenocysteinyl-tRNA(Sec) biosynthesis

This protein is involved in step 1 of the subpathway that synthesizes L-seryl-tRNA(Sec) from L-serine and tRNA(Sec).
Proteins known to be involved in this subpathway in this organism are:
  1. Serine--tRNA ligase, cytoplasmic (SARS), Serine--tRNA ligase, mitochondrial (SARS2)
This subpathway is part of the pathway selenocysteinyl-tRNA(Sec) biosynthesis, which is itself part of Aminoacyl-tRNA biosynthesis.
View all proteins of this organism that are known to be involved in the subpathway that synthesizes L-seryl-tRNA(Sec) from L-serine and tRNA(Sec), the pathway selenocysteinyl-tRNA(Sec) biosynthesis and in Aminoacyl-tRNA biosynthesis.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Binding sitei345ATP; via amide nitrogen and carbonyl oxygenBy similarity1
Binding sitei352L-serineBy similarity1
Binding sitei453L-serineBy similarity1

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Nucleotide bindingi330 – 332ATPBy similarity3
Nucleotide bindingi418 – 421ATPBy similarity4

GO - Molecular functioni

  • ATP binding Source: UniProtKB
  • RNA binding Source: UniProtKB
  • serine-tRNA ligase activity Source: UniProtKB

GO - Biological processi

Keywordsi

Molecular functionAminoacyl-tRNA synthetase, Ligase
Biological processProtein biosynthesis
LigandATP-binding, Nucleotide-binding

Enzyme and pathway databases

BRENDAi6.1.1.11 2681
ReactomeiR-HSA-379726 Mitochondrial tRNA aminoacylation
UniPathwayiUPA00906; UER00895

Names & Taxonomyi

Protein namesi
Recommended name:
Serine--tRNA ligase, mitochondrial (EC:6.1.1.11)
Alternative name(s):
SerRSmt
Seryl-tRNA synthetase
Short name:
SerRS
Seryl-tRNA(Ser/Sec) synthetase
Gene namesi
Name:SARS2
Synonyms:SARSM
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 19

Organism-specific databases

EuPathDBiHostDB:ENSG00000104835.14
HGNCiHGNC:17697 SARS2
MIMi612804 gene
neXtProtiNX_Q9NP81

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Mitochondrion

Pathology & Biotechi

Involvement in diseasei

Hyperuricemia, pulmonary hypertension, renal failure, and alkalosis syndrome (HUPRAS)1 Publication
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA 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.
See also OMIM:613845
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_065820390D → G in HUPRAS. 1 PublicationCorresponds to variant dbSNP:rs727502784Ensembl.1

Organism-specific databases

DisGeNETi54938
MalaCardsiSARS2
MIMi613845 phenotype
OpenTargetsiENSG00000104835
Orphaneti363694 Hyperuricemia-pulmonary hypertension-renal failure-alkalosis syndrome
PharmGKBiPA134899753

Polymorphism and mutation databases

BioMutaiSARS2
DMDMi23822219

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 34MitochondrionBy similarityAdd BLAST34
ChainiPRO_000003582235 – 518Serine--tRNA ligase, mitochondrialAdd BLAST484

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei110N6-acetyllysineBy similarity1
Modified residuei195N6-succinyllysineBy similarity1

Keywords - PTMi

Acetylation

Proteomic databases

EPDiQ9NP81
MaxQBiQ9NP81
PaxDbiQ9NP81
PeptideAtlasiQ9NP81
PRIDEiQ9NP81

PTM databases

iPTMnetiQ9NP81
PhosphoSitePlusiQ9NP81

Expressioni

Gene expression databases

BgeeiENSG00000104835
CleanExiHS_SARS2
ExpressionAtlasiQ9NP81 baseline and differential
GenevisibleiQ9NP81 HS

Organism-specific databases

HPAiHPA052730
HPA056957

Interactioni

Subunit structurei

Homodimer. The tRNA molecule probably binds across the dimer.By similarity

Protein-protein interaction databases

BioGridi12027834 interactors.
IntActiQ9NP81 17 interactors.
MINTiQ9NP81
STRINGi9606.ENSP00000472847

Structurei

3D structure databases

ProteinModelPortaliQ9NP81
SMRiQ9NP81
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni299 – 301L-serine bindingBy similarity3

Domaini

Consists of two distinct domains, a catalytic core and a N-terminal extension that is involved in tRNA binding.By similarity

Sequence similaritiesi

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG2509 Eukaryota
COG0172 LUCA
GeneTreeiENSGT00790000123121
HOGENOMiHOG000035937
HOVERGENiHBG023869
InParanoidiQ9NP81
KOiK01875
OMAiDTGKEPW
OrthoDBiEOG091G05BT
PhylomeDBiQ9NP81

Family and domain databases

CDDicd00770 SerRS_core, 1 hit
InterProiView protein in InterPro
IPR002314 aa-tRNA-synt_IIb
IPR006195 aa-tRNA-synth_II
IPR002317 Ser-tRNA-ligase_type_1
IPR033729 SerRS_core
IPR010978 tRNA-bd_arm
PANTHERiPTHR11778 PTHR11778, 1 hit
PfamiView protein in Pfam
PF00587 tRNA-synt_2b, 1 hit
PIRSFiPIRSF001529 Ser-tRNA-synth_IIa, 1 hit
PRINTSiPR00981 TRNASYNTHSER
SUPFAMiSSF46589 SSF46589, 1 hit
TIGRFAMsiTIGR00414 serS, 1 hit
PROSITEiView protein in PROSITE
PS50862 AA_TRNA_LIGASE_II, 1 hit

Sequences (2)i

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: Q9NP81-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide

        10         20         30         40         50
MAASMARRLW PLLTRRGFRP RGGCISNDSP RRSFTTEKRN RNLLYEYARE
60 70 80 90 100
GYSALPQLDI ERFCACPEEA AHALELRKGE LRSADLPAII STWQELRQLQ
110 120 130 140 150
EQIRSLEEEK AAVTEAVRAL LANQDSGEVQ QDPKYQGLRA RGREIRKELV
160 170 180 190 200
HLYPREAQLE EQFYLQALKL PNQTHPDVPV GDESQARVLH MVGDKPVFSF
210 220 230 240 250
QPRGHLEIGE KLDIIRQKRL SHVSGHRSYY LRGAGALLQH GLVNFTFNKL
260 270 280 290 300
LRRGFTPMTV PDLLRGAVFE GCGMTPNANP SQIYNIDPAR FKDLNLAGTA
310 320 330 340 350
EVGLAGYFMD HTVAFRDLPV RMVCSSTCYR AETNTGQEPR GLYRVHHFTK
360 370 380 390 400
VEMFGVTGPG LEQSSQLLEE FLSLQMEILT ELGLHFRVLD MPTQELGLPA
410 420 430 440 450
YRKFDIEAWM PGRGRFGEVT SASNCTDFQS RRLHIMFQTE AGELQFAHTV
460 470 480 490 500
NATACAVPRL LIALLESNQQ KDGSVLVPPA LQSYLGTDRI TAPTHVPLQY
510
IGPNQPRKPG LPGQPAVS
Length:518
Mass (Da):58,283
Last modified:October 1, 2000 - v1
Checksum:iC6516A9527B34EB7
GO
Isoform 2 (identifier: Q9NP81-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     132-155: DPKYQGLRARGREIRKELVHLYPR → VRLDPGAGSIFGPTFLPFPGQLSLLV

Note: No experimental confirmation available.
Show »
Length:520
Mass (Da):58,030
Checksum:i1CB28DA9FBFEED8D
GO

Sequence cautioni

The sequence AAH01020 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.Curated

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_05264535T → A. Corresponds to variant dbSNP:rs34264048Ensembl.1
Natural variantiVAR_05264683S → L. Corresponds to variant dbSNP:rs34050897Ensembl.1
Natural variantiVAR_065820390D → G in HUPRAS. 1 PublicationCorresponds to variant dbSNP:rs727502784Ensembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_043020132 – 155DPKYQ…HLYPR → VRLDPGAGSIFGPTFLPFPG QLSLLV in isoform 2. 1 PublicationAdd BLAST24

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AB029948 mRNA Translation: BAA99557.1
AK000457 mRNA Translation: BAA91176.1
AK293414 mRNA Translation: BAG56921.1
AC011455 Genomic DNA No translation available.
BC001020 mRNA Translation: AAH01020.2 Different initiation.
BC042912 mRNA Translation: AAH42912.1
CCDSiCCDS33017.1 [Q9NP81-1]
CCDS54265.1 [Q9NP81-2]
RefSeqiNP_001139373.1, NM_001145901.1 [Q9NP81-2]
NP_060297.1, NM_017827.3 [Q9NP81-1]
UniGeneiHs.709416

Genome annotation databases

EnsembliENST00000221431; ENSP00000221431; ENSG00000104835 [Q9NP81-1]
ENST00000600042; ENSP00000472847; ENSG00000104835 [Q9NP81-2]
ENST00000634687; ENSP00000489539; ENSG00000283104 [Q9NP81-1]
ENST00000635245; ENSP00000489453; ENSG00000283104 [Q9NP81-2]
GeneIDi54938
KEGGihsa:54938
UCSCiuc002oka.3 human [Q9NP81-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Similar proteinsi

Entry informationi

Entry nameiSYSM_HUMAN
AccessioniPrimary (citable) accession number: Q9NP81
Secondary accession number(s): A6NHW7, B4DE10, Q9BVP3
Entry historyiIntegrated into UniProtKB/Swiss-Prot: October 10, 2002
Last sequence update: October 1, 2000
Last modified: March 28, 2018
This is version 159 of the entry and version 1 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome