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Protein

Asparagine synthetase [glutamine-hydrolyzing]

Gene

ASNS

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

Functioni

Catalytic activityi

ATP + L-aspartate + L-glutamine + H2O = AMP + diphosphate + L-asparagine + L-glutamate.1 Publication

Pathwayi: L-asparagine biosynthesis

This protein is involved in step 1 of the subpathway that synthesizes L-asparagine from L-aspartate (L-Gln route).
Proteins known to be involved in this subpathway in this organism are:
  1. Asparagine synthetase [glutamine-hydrolyzing] (ASNS)
This subpathway is part of the pathway L-asparagine biosynthesis, which is itself part of Amino-acid biosynthesis.
View all proteins of this organism that are known to be involved in the subpathway that synthesizes L-asparagine from L-aspartate (L-Gln route), the pathway L-asparagine biosynthesis and in Amino-acid biosynthesis.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Active sitei2For GATase activity2 Publications1
Binding sitei97GlutamineBy similarity1
Binding sitei256ATP; via carbonyl oxygenBy similarity1
Binding sitei288ATP; via amide nitrogen and carbonyl oxygenBy similarity1
Sitei365Important for beta-aspartyl-AMP intermediate formationBy similarity1

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Nucleotide bindingi363 – 364ATPBy similarity2

GO - Molecular functioni

  • asparagine synthase (glutamine-hydrolyzing) activity Source: UniProtKB
  • ATP binding Source: UniProtKB-KW
  • cofactor binding Source: Ensembl
  • protein homodimerization activity Source: GO_Central

GO - Biological processi

Keywordsi

Molecular functionLigase
Biological processAmino-acid biosynthesis, Asparagine biosynthesis
LigandATP-binding, Nucleotide-binding

Enzyme and pathway databases

BRENDAi6.3.5.4 2681
ReactomeiR-HSA-380994 ATF4 activates genes
R-HSA-70614 Amino acid synthesis and interconversion (transamination)
SIGNORiP08243
UniPathwayi
UPA00134;UER00195

Protein family/group databases

MEROPSiC44.974

Names & Taxonomyi

Protein namesi
Recommended name:
Asparagine synthetase [glutamine-hydrolyzing] (EC:6.3.5.4)
Alternative name(s):
Cell cycle control protein TS11
Glutamine-dependent asparagine synthetase
Gene namesi
Name:ASNS
Synonyms:TS11
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 7

Organism-specific databases

EuPathDBiHostDB:ENSG00000070669.16
HGNCiHGNC:753 ASNS
MIMi108370 gene
neXtProtiNX_P08243

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

Pathology & Biotechi

Involvement in diseasei

Asparagine synthetase deficiency (ASNSD)1 Publication
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionAn inborn error of asparagine biosynthesis that results in a severe neurologic disorder characterized by microcephaly, severely delayed psychomotor development, progressive encephalopathy, cortical atrophy, and seizure or hyperekplexic activity.
See also OMIM:615574
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_0708966A → E in ASNSD; dramatic reduction in protein abundance. 1 PublicationCorresponds to variant dbSNP:rs398122975EnsemblClinVar.1
Natural variantiVAR_070897362F → V in ASNSD; dramatic reduction in protein abundance. 1 PublicationCorresponds to variant dbSNP:rs398122973EnsemblClinVar.1
Natural variantiVAR_070898550R → C in ASNSD; increases level of protein abundance. 1 PublicationCorresponds to variant dbSNP:rs398122974EnsemblClinVar.1

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Mutagenesisi2C → A: Loss of the glutamine-dependent asparagine synthetase activity, while the ammonia-dependent activity remained unaffected. 1 Publication1

Keywords - Diseasei

Disease mutation, Mental retardation

Organism-specific databases

DisGeNETi440
MalaCardsiASNS
MIMi615574 phenotype
OpenTargetsiENSG00000070669
Orphaneti391376 Congenital microcephaly-severe encephalopathy-progressive cerebral atrophy syndrome
PharmGKBiPA25052

Chemistry databases

ChEMBLiCHEMBL3120
DrugBankiDB00171 Adenosine triphosphate
DB00174 L-Asparagine
DB00128 L-Aspartic Acid
DB00142 L-Glutamic Acid
DB00130 L-Glutamine

Polymorphism and mutation databases

BioMutaiASNS
DMDMi13432102

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Initiator methionineiRemovedBy similarity
ChainiPRO_00000569102 – 561Asparagine synthetase [glutamine-hydrolyzing]Add BLAST560

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei385N6-acetyllysineCombined sources1
Modified residuei545PhosphothreonineCombined sources1
Modified residuei557PhosphoserineCombined sources1

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

EPDiP08243
MaxQBiP08243
PaxDbiP08243
PeptideAtlasiP08243
PRIDEiP08243
ProteomicsDBi52098

PTM databases

iPTMnetiP08243
PhosphoSitePlusiP08243
SwissPalmiP08243

Expressioni

Gene expression databases

BgeeiENSG00000070669 Expressed in 227 organ(s), highest expression level in cerebellar hemisphere
CleanExiHS_ASNS
ExpressionAtlasiP08243 baseline and differential
GenevisibleiP08243 HS

Organism-specific databases

HPAiHPA004924
HPA029318
HPA064737

Interactioni

Binary interactionsi

GO - Molecular functioni

Protein-protein interaction databases

BioGridi106932, 87 interactors
IntActiP08243, 12 interactors
MINTiP08243
STRINGi9606.ENSP00000175506

Chemistry databases

BindingDBiP08243

Structurei

3D structure databases

ProteinModelPortaliP08243
SMRiP08243
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini2 – 191Glutamine amidotransferase type-2PROSITE-ProRule annotationAdd BLAST190
Domaini213 – 536Asparagine synthetaseAdd BLAST324

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni49 – 53Glutamine bindingBy similarity5
Regioni75 – 77Glutamine bindingBy similarity3

Keywords - Domaini

Glutamine amidotransferase

Phylogenomic databases

eggNOGiKOG0571 Eukaryota
COG0367 LUCA
GeneTreeiENSGT00390000001994
HOGENOMiHOG000027493
HOVERGENiHBG003103
InParanoidiP08243
KOiK01953
OMAiLFGYKYT
OrthoDBiEOG091G13BD
PhylomeDBiP08243
TreeFamiTF300603

Family and domain databases

CDDicd01991 Asn_Synthase_B_C, 1 hit
cd00712 AsnB, 1 hit
Gene3Di3.40.50.620, 1 hit
3.60.20.10, 1 hit
InterProiView protein in InterPro
IPR006426 Asn_synth_AEB
IPR001962 Asn_synthase
IPR033738 AsnB_N
IPR017932 GATase_2_dom
IPR029055 Ntn_hydrolases_N
IPR014729 Rossmann-like_a/b/a_fold
PfamiView protein in Pfam
PF00733 Asn_synthase, 1 hit
PF13537 GATase_7, 1 hit
PIRSFiPIRSF001589 Asn_synthetase_glu-h, 1 hit
SUPFAMiSSF56235 SSF56235, 1 hit
TIGRFAMsiTIGR01536 asn_synth_AEB, 1 hit
PROSITEiView protein in PROSITE
PS51278 GATASE_TYPE_2, 1 hit

Sequences (3+)i

Sequence statusi: Complete.

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

This entry describes 3 isoformsi produced by alternative splicing. AlignAdd to basket

This entry has 3 described isoforms and 7 potential isoforms that are computationally mapped.Show allAlign All

Isoform 1 (identifier: P08243-1) [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
MCGIWALFGS DDCLSVQCLS AMKIAHRGPD AFRFENVNGY TNCCFGFHRL
60 70 80 90 100
AVVDPLFGMQ PIRVKKYPYL WLCYNGEIYN HKKMQQHFEF EYQTKVDGEI
110 120 130 140 150
ILHLYDKGGI EQTICMLDGV FAFVLLDTAN KKVFLGRDTY GVRPLFKAMT
160 170 180 190 200
EDGFLAVCSE AKGLVTLKHS ATPFLKVEPF LPGHYEVLDL KPNGKVASVE
210 220 230 240 250
MVKYHHCRDV PLHALYDNVE KLFPGFEIET VKNNLRILFN NAVKKRLMTD
260 270 280 290 300
RRIGCLLSGG LDSSLVAATL LKQLKEAQVQ YPLQTFAIGM EDSPDLLAAR
310 320 330 340 350
KVADHIGSEH YEVLFNSEEG IQALDEVIFS LETYDITTVR ASVGMYLISK
360 370 380 390 400
YIRKNTDSVV IFSGEGSDEL TQGYIYFHKA PSPEKAEEES ERLLRELYLF
410 420 430 440 450
DVLRADRTTA AHGLELRVPF LDHRFSSYYL SLPPEMRIPK NGIEKHLLRE
460 470 480 490 500
TFEDSNLIPK EILWRPKEAF SDGITSVKNS WFKILQEYVE HQVDDAMMAN
510 520 530 540 550
AAQKFPFNTP KTKEGYYYRQ VFERHYPGRA DWLSHYWMPK WINATDPSAR
560
TLTHYKSAVK A
Length:561
Mass (Da):64,370
Last modified:January 23, 2007 - v4
Checksum:i3E9B75E21D799FBE
GO
Isoform 2 (identifier: P08243-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-21: Missing.

Note: No experimental confirmation available.
Show »
Length:540
Mass (Da):62,168
Checksum:i7531F53E232D5AB0
GO
Isoform 3 (identifier: P08243-3) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-83: Missing.

Note: No experimental confirmation available.
Show »
Length:478
Mass (Da):54,818
Checksum:iF8AE4D4375F38522
GO

Computationally mapped potential isoform sequencesi

There are 7 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
C9JT45C9JT45_HUMAN
Asparagine synthetase [glutamine-hy...
ASNS
162Annotation score:
C9J057C9J057_HUMAN
Asparagine synthetase [glutamine-hy...
ASNS
214Annotation score:
C9JM09C9JM09_HUMAN
Asparagine synthetase [glutamine-hy...
ASNS
118Annotation score:
F8WEJ5F8WEJ5_HUMAN
Asparagine synthetase [glutamine-hy...
ASNS
401Annotation score:
C9JLN6C9JLN6_HUMAN
Asparagine synthetase [glutamine-hy...
ASNS
63Annotation score:
C9J605C9J605_HUMAN
Asparagine synthetase [glutamine-hy...
ASNS
32Annotation score:
C9JLA3C9JLA3_HUMAN
Asparagine synthetase [glutamine-hy...
ASNS
28Annotation score:

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti333 – 343TYDITTVRASV → LMTLQQFVLRI in AAA36781 (PubMed:3470743).CuratedAdd BLAST11
Sequence conflicti353 – 360RKNTDSVV → GRTQIAWL in AAA36781 (PubMed:3470743).Curated8
Sequence conflicti426S → F (PubMed:2886907).Curated1
Sequence conflicti426S → F (PubMed:2565875).Curated1
Sequence conflicti462I → V in BAG63553 (PubMed:14702039).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_0708966A → E in ASNSD; dramatic reduction in protein abundance. 1 PublicationCorresponds to variant dbSNP:rs398122975EnsemblClinVar.1
Natural variantiVAR_023443210V → E5 PublicationsCorresponds to variant dbSNP:rs1049674Ensembl.1
Natural variantiVAR_070897362F → V in ASNSD; dramatic reduction in protein abundance. 1 PublicationCorresponds to variant dbSNP:rs398122973EnsemblClinVar.1
Natural variantiVAR_070898550R → C in ASNSD; increases level of protein abundance. 1 PublicationCorresponds to variant dbSNP:rs398122974EnsemblClinVar.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_0458171 – 83Missing in isoform 3. 1 PublicationAdd BLAST83
Alternative sequenceiVSP_0458181 – 21Missing in isoform 2. 1 PublicationAdd BLAST21

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
M27396 mRNA Translation: AAA51789.1
L35946
, L35936, L35937, L35938, L35939, L35940, L35941, L35942, L35943, L35944, L35945 Genomic DNA Translation: AAA52756.1
BT007113 mRNA Translation: AAP35777.1
AK302189 mRNA Translation: BAG63553.1
AK316224 mRNA Translation: BAH14595.1
AC005326 Genomic DNA No translation available.
AC079781 Genomic DNA Translation: AAQ96856.1
CH236949 Genomic DNA Translation: EAL24115.1
CH471091 Genomic DNA Translation: EAW76730.1
CH471091 Genomic DNA Translation: EAW76723.1
CH471091 Genomic DNA Translation: EAW76731.1
CH471091 Genomic DNA Translation: EAW76732.1
CH471091 Genomic DNA Translation: EAW76733.1
BC008723 mRNA Translation: AAH08723.1
BC014621 mRNA Translation: AAH14621.1
M15798 mRNA Translation: AAA36781.1
M27054 Genomic DNA Translation: AAA63266.1
CCDSiCCDS55131.1 [P08243-3]
CCDS55132.1 [P08243-2]
CCDS5652.1 [P08243-1]
PIRiA27062 AJHUN1
RefSeqiNP_001171546.1, NM_001178075.1 [P08243-2]
NP_001171547.1, NM_001178076.1 [P08243-3]
NP_001171548.1, NM_001178077.1 [P08243-3]
NP_001664.3, NM_001673.4 [P08243-1]
NP_597680.2, NM_133436.3 [P08243-1]
NP_899199.2, NM_183356.3 [P08243-1]
UniGeneiHs.489207

Genome annotation databases

EnsembliENST00000175506; ENSP00000175506; ENSG00000070669 [P08243-1]
ENST00000394308; ENSP00000377845; ENSG00000070669 [P08243-1]
ENST00000394309; ENSP00000377846; ENSG00000070669 [P08243-1]
ENST00000422745; ENSP00000414901; ENSG00000070669 [P08243-2]
ENST00000437628; ENSP00000414379; ENSG00000070669 [P08243-3]
ENST00000444334; ENSP00000406994; ENSG00000070669 [P08243-2]
ENST00000455086; ENSP00000408472; ENSG00000070669 [P08243-3]
GeneIDi440
KEGGihsa:440
UCSCiuc003uot.5 human [P08243-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Cross-referencesi

Web resourcesi

Atlas of Genetics and Cytogenetics in Oncology and Haematology

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
M27396 mRNA Translation: AAA51789.1
L35946
, L35936, L35937, L35938, L35939, L35940, L35941, L35942, L35943, L35944, L35945 Genomic DNA Translation: AAA52756.1
BT007113 mRNA Translation: AAP35777.1
AK302189 mRNA Translation: BAG63553.1
AK316224 mRNA Translation: BAH14595.1
AC005326 Genomic DNA No translation available.
AC079781 Genomic DNA Translation: AAQ96856.1
CH236949 Genomic DNA Translation: EAL24115.1
CH471091 Genomic DNA Translation: EAW76730.1
CH471091 Genomic DNA Translation: EAW76723.1
CH471091 Genomic DNA Translation: EAW76731.1
CH471091 Genomic DNA Translation: EAW76732.1
CH471091 Genomic DNA Translation: EAW76733.1
BC008723 mRNA Translation: AAH08723.1
BC014621 mRNA Translation: AAH14621.1
M15798 mRNA Translation: AAA36781.1
M27054 Genomic DNA Translation: AAA63266.1
CCDSiCCDS55131.1 [P08243-3]
CCDS55132.1 [P08243-2]
CCDS5652.1 [P08243-1]
PIRiA27062 AJHUN1
RefSeqiNP_001171546.1, NM_001178075.1 [P08243-2]
NP_001171547.1, NM_001178076.1 [P08243-3]
NP_001171548.1, NM_001178077.1 [P08243-3]
NP_001664.3, NM_001673.4 [P08243-1]
NP_597680.2, NM_133436.3 [P08243-1]
NP_899199.2, NM_183356.3 [P08243-1]
UniGeneiHs.489207

3D structure databases

ProteinModelPortaliP08243
SMRiP08243
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi106932, 87 interactors
IntActiP08243, 12 interactors
MINTiP08243
STRINGi9606.ENSP00000175506

Chemistry databases

BindingDBiP08243
ChEMBLiCHEMBL3120
DrugBankiDB00171 Adenosine triphosphate
DB00174 L-Asparagine
DB00128 L-Aspartic Acid
DB00142 L-Glutamic Acid
DB00130 L-Glutamine

Protein family/group databases

MEROPSiC44.974

PTM databases

iPTMnetiP08243
PhosphoSitePlusiP08243
SwissPalmiP08243

Polymorphism and mutation databases

BioMutaiASNS
DMDMi13432102

Proteomic databases

EPDiP08243
MaxQBiP08243
PaxDbiP08243
PeptideAtlasiP08243
PRIDEiP08243
ProteomicsDBi52098

Protocols and materials databases

DNASUi440
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000175506; ENSP00000175506; ENSG00000070669 [P08243-1]
ENST00000394308; ENSP00000377845; ENSG00000070669 [P08243-1]
ENST00000394309; ENSP00000377846; ENSG00000070669 [P08243-1]
ENST00000422745; ENSP00000414901; ENSG00000070669 [P08243-2]
ENST00000437628; ENSP00000414379; ENSG00000070669 [P08243-3]
ENST00000444334; ENSP00000406994; ENSG00000070669 [P08243-2]
ENST00000455086; ENSP00000408472; ENSG00000070669 [P08243-3]
GeneIDi440
KEGGihsa:440
UCSCiuc003uot.5 human [P08243-1]

Organism-specific databases

CTDi440
DisGeNETi440
EuPathDBiHostDB:ENSG00000070669.16
GeneCardsiASNS
HGNCiHGNC:753 ASNS
HPAiHPA004924
HPA029318
HPA064737
MalaCardsiASNS
MIMi108370 gene
615574 phenotype
neXtProtiNX_P08243
OpenTargetsiENSG00000070669
Orphaneti391376 Congenital microcephaly-severe encephalopathy-progressive cerebral atrophy syndrome
PharmGKBiPA25052
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG0571 Eukaryota
COG0367 LUCA
GeneTreeiENSGT00390000001994
HOGENOMiHOG000027493
HOVERGENiHBG003103
InParanoidiP08243
KOiK01953
OMAiLFGYKYT
OrthoDBiEOG091G13BD
PhylomeDBiP08243
TreeFamiTF300603

Enzyme and pathway databases

UniPathwayi
UPA00134;UER00195

BRENDAi6.3.5.4 2681
ReactomeiR-HSA-380994 ATF4 activates genes
R-HSA-70614 Amino acid synthesis and interconversion (transamination)
SIGNORiP08243

Miscellaneous databases

ChiTaRSiASNS human
GenomeRNAii440
PROiPR:P08243
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000070669 Expressed in 227 organ(s), highest expression level in cerebellar hemisphere
CleanExiHS_ASNS
ExpressionAtlasiP08243 baseline and differential
GenevisibleiP08243 HS

Family and domain databases

CDDicd01991 Asn_Synthase_B_C, 1 hit
cd00712 AsnB, 1 hit
Gene3Di3.40.50.620, 1 hit
3.60.20.10, 1 hit
InterProiView protein in InterPro
IPR006426 Asn_synth_AEB
IPR001962 Asn_synthase
IPR033738 AsnB_N
IPR017932 GATase_2_dom
IPR029055 Ntn_hydrolases_N
IPR014729 Rossmann-like_a/b/a_fold
PfamiView protein in Pfam
PF00733 Asn_synthase, 1 hit
PF13537 GATase_7, 1 hit
PIRSFiPIRSF001589 Asn_synthetase_glu-h, 1 hit
SUPFAMiSSF56235 SSF56235, 1 hit
TIGRFAMsiTIGR01536 asn_synth_AEB, 1 hit
PROSITEiView protein in PROSITE
PS51278 GATASE_TYPE_2, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiASNS_HUMAN
AccessioniPrimary (citable) accession number: P08243
Secondary accession number(s): A4D1I8
, B4DXZ1, B7ZAA9, D6W5R3, E9PCI3, E9PCX6, P08184, Q15666, Q549T9, Q96HD0
Entry historyiIntegrated into UniProtKB/Swiss-Prot: August 1, 1988
Last sequence update: January 23, 2007
Last modified: November 7, 2018
This is version 205 of the entry and version 4 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

Documents

  1. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  2. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  3. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  4. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  5. Human chromosome 7
    Human chromosome 7: entries, gene names and cross-references to MIM
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