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Protein

Aminoacyl tRNA synthase complex-interacting multifunctional protein 1

Gene

AIMP1

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

Functioni

Non-catalytic component of the multisynthase complex. Stimulates the catalytic activity of cytoplasmic arginyl-tRNA synthase (PubMed:10358004). Binds tRNA. Possesses inflammatory cytokine activity (PubMed:11306575). Negatively regulates TGF-beta signaling through stabilization of SMURF2 by binding to SMURF2 and inhibiting its SMAD7-mediated degradation (By similarity). Involved in glucose homeostasis through induction of glucagon secretion at low glucose levels (By similarity). Promotes dermal fibroblast proliferation and wound repair (PubMed:16472771). Regulates KDELR1-mediated retention of HSP90B1/gp96 in the endoplasmic reticulum (By similarity). Plays a role in angiogenesis by inducing endothelial cell migration at low concentrations and endothelian cell apoptosis at high concentrations (PubMed:12237313). Induces maturation of dendritic cells and monocyte cell adhesion (PubMed:11818442). Modulates endothelial cell responses by degrading HIF-1A through interaction with PSMA7 (PubMed:19362550).By similarity6 Publications

GO - Molecular functioni

  • cytokine activity Source: HGNC
  • GTPase binding Source: UniProtKB
  • protein homodimerization activity Source: HGNC
  • tRNA binding Source: HGNC

GO - Biological processi

Keywordsi

Molecular functionCytokine, RNA-binding, tRNA-binding
Biological processAngiogenesis, Apoptosis, Cell adhesion, Inflammatory response, Protein biosynthesis

Enzyme and pathway databases

ReactomeiR-HSA-2408517 SeMet incorporation into proteins
R-HSA-379716 Cytosolic tRNA aminoacylation
SIGNORiQ12904

Names & Taxonomyi

Protein namesi
Recommended name:
Aminoacyl tRNA synthase complex-interacting multifunctional protein 1
Alternative name(s):
Multisynthase complex auxiliary component p43
Cleaved into the following chain:
Alternative name(s):
Endothelial monocyte-activating polypeptide II
Short name:
EMAP-II
Small inducible cytokine subfamily E member 1
Gene namesi
Name:AIMP1
Synonyms:EMAP2, SCYE1
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 4

Organism-specific databases

EuPathDBiHostDB:ENSG00000164022.16
HGNCiHGNC:10648 AIMP1
MIMi603605 gene
neXtProtiNX_Q12904

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

Cytoplasm, Endoplasmic reticulum, Golgi apparatus, Nucleus, Secreted

Pathology & Biotechi

Involvement in diseasei

Leukodystrophy, hypomyelinating, 3 (HLD3)1 Publication
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA severe autosomal recessive hypomyelinating leukodystrophy characterized by early infantile onset of global developmental delay, lack of development, lack of speech acquisition, and peripheral spasticity associated with decreased myelination in the central nervous system.
See also OMIM:260600

Keywords - Diseasei

Leukodystrophy

Organism-specific databases

DisGeNETi9255
MalaCardsiAIMP1
MIMi260600 phenotype
OpenTargetsiENSG00000164022
Orphaneti88616 Autosomal recessive non-syndromic intellectual disability
280293 Pelizaeus-Merzbacher-like disease due to AIMP1 mutation
PharmGKBiPA35578

Polymorphism and mutation databases

BioMutaiAIMP1
DMDMi85700432

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Initiator methionineiRemovedCombined sources1 Publication
ChainiPRO_00002233942 – 312Aminoacyl tRNA synthase complex-interacting multifunctional protein 1Add BLAST311
ChainiPRO_0000019242147 – 312Endothelial monocyte-activating polypeptide 2Add BLAST166

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei2N-acetylalanineCombined sources1 Publication1
Cross-linki137Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in SUMO1)Combined sources
Modified residuei140PhosphoserineCombined sources1
Modified residuei269N6-succinyllysineBy similarity1

Post-translational modificationi

Cleaved by caspase-7 in response to apoptosis to produce EMAP-II.2 Publications

Keywords - PTMi

Acetylation, Isopeptide bond, Phosphoprotein, Ubl conjugation

Proteomic databases

EPDiQ12904
MaxQBiQ12904
PaxDbiQ12904
PeptideAtlasiQ12904
PRIDEiQ12904
ProteomicsDBi59011
59012 [Q12904-2]

PTM databases

iPTMnetiQ12904
PhosphoSitePlusiQ12904

Expressioni

Gene expression databases

BgeeiENSG00000164022 Expressed in 233 organ(s), highest expression level in amniotic fluid
CleanExiHS_SCYE1
ExpressionAtlasiQ12904 baseline and differential
GenevisibleiQ12904 HS

Organism-specific databases

HPAiCAB017618
HPA018476

Interactioni

Subunit structurei

Homodimer. Part of the multisynthetase complex (MSC), a multisubunit complex that groups tRNA ligases for Arg (RARS), Asp (DARS), Gln (QARS), Ile (IARS), Leu (LARS), Lys (KARS), Met (MARS) the bifunctional ligase for Glu and Pro (EPRS) and the auxiliary subunits AIMP1/p43, AIMP2/p38 and EEF1E1/p18 (PubMed:24312579, PubMed:19131329, PubMed:19289464). Interacts (via N-terminus) with RARS (via N-terminus) (PubMed:10358004, PubMed:17443684). Part of a complex composed of RARS, QARS and AIMP1 (PubMed:25288775). Interacts (via C-terminus) with SMURF2. Interacts (via N-terminus) with HSP90B1/gp96 (via C-terminus) (By similarity). Interacts with PSMA7 (PubMed:19362550). Interacts with TARSL2 (PubMed:24312579).By similarity8 Publications

Binary interactionsi

GO - Molecular functioni

Protein-protein interaction databases

BioGridi114679, 115 interactors
CORUMiQ12904
IntActiQ12904, 23 interactors
MINTiQ12904
STRINGi9606.ENSP00000378191

Structurei

Secondary structure

1312
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details

3D structure databases

ProteinModelPortaliQ12904
SMRiQ12904
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiQ12904

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini151 – 252tRNA-bindingPROSITE-ProRule annotationAdd BLAST102

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni6 – 46Required for fibroblast proliferationAdd BLAST41
Regioni54 – 194Interaction with HSP90B1By similarityAdd BLAST141
Regioni101 – 114Required for endothelial cell deathAdd BLAST14
Regioni114 – 192Required for endothelial cell migrationAdd BLAST79

Phylogenomic databases

eggNOGiKOG2241 Eukaryota
COG0073 LUCA
GeneTreeiENSGT00930000150953
HOGENOMiHOG000230584
HOVERGENiHBG019053
InParanoidiQ12904
KOiK15437
OMAiNDQCVAT
OrthoDBiEOG091G0OTM
PhylomeDBiQ12904
TreeFamiTF324775

Family and domain databases

InterProiView protein in InterPro
IPR012340 NA-bd_OB-fold
IPR002547 tRNA-bd_dom
PfamiView protein in Pfam
PF01588 tRNA_bind, 1 hit
SUPFAMiSSF50249 SSF50249, 1 hit
PROSITEiView protein in PROSITE
PS50886 TRBD, 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

This entry has 2 described isoforms and 1 potential isoform that is computationally mapped.Show allAlign All

Isoform 1 (identifier: Q12904-1) [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
MANNDAVLKR LEQKGAEADQ IIEYLKQQVS LLKEKAILQA TLREEKKLRV
60 70 80 90 100
ENAKLKKEIE ELKQELIQAE IQNGVKQIPF PSGTPLHANS MVSENVIQST
110 120 130 140 150
AVTTVSSGTK EQIKGGTGDE KKAKEKIEKK GEKKEKKQQS IAGSADSKPI
160 170 180 190 200
DVSRLDLRIG CIITARKHPD ADSLYVEEVD VGEIAPRTVV SGLVNHVPLE
210 220 230 240 250
QMQNRMVILL CNLKPAKMRG VLSQAMVMCA SSPEKIEILA PPNGSVPGDR
260 270 280 290 300
ITFDAFPGEP DKELNPKKKI WEQIQPDLHT NDECVATYKG VPFEVKGKGV
310
CRAQTMSNSG IK
Length:312
Mass (Da):34,353
Last modified:January 24, 2006 - v2
Checksum:i5F0BF73E58810C60
GO
Isoform 2 (identifier: Q12904-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-1: M → MLPAVAVSEPVVLRFMIFCRLLAKM

Show »
Length:336
Mass (Da):37,039
Checksum:iC4D804F0180FD7D4
GO

Computationally mapped potential isoform sequencesi

There is 1 potential isoform mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
D6R937D6R937_HUMAN
Aminoacyl tRNA synthase complex-int...
AIMP1
150Annotation score:

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti138Q → R in BAG64889 (PubMed:14702039).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_02521279P → A1 PublicationCorresponds to variant dbSNP:rs1134648Ensembl.1
Natural variantiVAR_029156104T → A. Corresponds to variant dbSNP:rs113844295EnsemblClinVar.1
Natural variantiVAR_050124117T → A. Corresponds to variant dbSNP:rs2230255Ensembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_0421971M → MLPAVAVSEPVVLRFMIFCR LLAKM in isoform 2. 1 Publication1 Publication1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
U10117 mRNA Translation: AAA62202.1
CR542281 mRNA Translation: CAG47076.1
AK095951 mRNA Translation: BAG53174.1
AK303965 mRNA Translation: BAG64889.1
BC014051 mRNA Translation: AAH14051.1
CCDSiCCDS3674.1 [Q12904-1]
CCDS47121.1 [Q12904-2]
PIRiB55053
RefSeqiNP_001135887.1, NM_001142415.1 [Q12904-1]
NP_001135888.1, NM_001142416.1 [Q12904-2]
NP_004748.2, NM_004757.3 [Q12904-1]
XP_016864324.1, XM_017008835.1 [Q12904-1]
XP_016864325.1, XM_017008836.1 [Q12904-1]
UniGeneiHs.591680

Genome annotation databases

EnsembliENST00000358008; ENSP00000350699; ENSG00000164022 [Q12904-1]
ENST00000394701; ENSP00000378191; ENSG00000164022 [Q12904-2]
ENST00000442366; ENSP00000405248; ENSG00000164022 [Q12904-1]
GeneIDi9255
KEGGihsa:9255
UCSCiuc003hyg.4 human [Q12904-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Similar proteinsi

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
U10117 mRNA Translation: AAA62202.1
CR542281 mRNA Translation: CAG47076.1
AK095951 mRNA Translation: BAG53174.1
AK303965 mRNA Translation: BAG64889.1
BC014051 mRNA Translation: AAH14051.1
CCDSiCCDS3674.1 [Q12904-1]
CCDS47121.1 [Q12904-2]
PIRiB55053
RefSeqiNP_001135887.1, NM_001142415.1 [Q12904-1]
NP_001135888.1, NM_001142416.1 [Q12904-2]
NP_004748.2, NM_004757.3 [Q12904-1]
XP_016864324.1, XM_017008835.1 [Q12904-1]
XP_016864325.1, XM_017008836.1 [Q12904-1]
UniGeneiHs.591680

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
1E7ZX-ray2.05A148-312[»]
1EUJX-ray1.80A/B147-312[»]
1FL0X-ray1.50A150-312[»]
4R3ZX-ray4.03A1-312[»]
ProteinModelPortaliQ12904
SMRiQ12904
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi114679, 115 interactors
CORUMiQ12904
IntActiQ12904, 23 interactors
MINTiQ12904
STRINGi9606.ENSP00000378191

PTM databases

iPTMnetiQ12904
PhosphoSitePlusiQ12904

Polymorphism and mutation databases

BioMutaiAIMP1
DMDMi85700432

Proteomic databases

EPDiQ12904
MaxQBiQ12904
PaxDbiQ12904
PeptideAtlasiQ12904
PRIDEiQ12904
ProteomicsDBi59011
59012 [Q12904-2]

Protocols and materials databases

DNASUi9255
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000358008; ENSP00000350699; ENSG00000164022 [Q12904-1]
ENST00000394701; ENSP00000378191; ENSG00000164022 [Q12904-2]
ENST00000442366; ENSP00000405248; ENSG00000164022 [Q12904-1]
GeneIDi9255
KEGGihsa:9255
UCSCiuc003hyg.4 human [Q12904-1]

Organism-specific databases

CTDi9255
DisGeNETi9255
EuPathDBiHostDB:ENSG00000164022.16
GeneCardsiAIMP1
HGNCiHGNC:10648 AIMP1
HPAiCAB017618
HPA018476
MalaCardsiAIMP1
MIMi260600 phenotype
603605 gene
neXtProtiNX_Q12904
OpenTargetsiENSG00000164022
Orphaneti88616 Autosomal recessive non-syndromic intellectual disability
280293 Pelizaeus-Merzbacher-like disease due to AIMP1 mutation
PharmGKBiPA35578
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG2241 Eukaryota
COG0073 LUCA
GeneTreeiENSGT00930000150953
HOGENOMiHOG000230584
HOVERGENiHBG019053
InParanoidiQ12904
KOiK15437
OMAiNDQCVAT
OrthoDBiEOG091G0OTM
PhylomeDBiQ12904
TreeFamiTF324775

Enzyme and pathway databases

ReactomeiR-HSA-2408517 SeMet incorporation into proteins
R-HSA-379716 Cytosolic tRNA aminoacylation
SIGNORiQ12904

Miscellaneous databases

ChiTaRSiAIMP1 human
EvolutionaryTraceiQ12904
GeneWikiiSCYE1
GenomeRNAii9255
PROiPR:Q12904
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000164022 Expressed in 233 organ(s), highest expression level in amniotic fluid
CleanExiHS_SCYE1
ExpressionAtlasiQ12904 baseline and differential
GenevisibleiQ12904 HS

Family and domain databases

InterProiView protein in InterPro
IPR012340 NA-bd_OB-fold
IPR002547 tRNA-bd_dom
PfamiView protein in Pfam
PF01588 tRNA_bind, 1 hit
SUPFAMiSSF50249 SSF50249, 1 hit
PROSITEiView protein in PROSITE
PS50886 TRBD, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiAIMP1_HUMAN
AccessioniPrimary (citable) accession number: Q12904
Secondary accession number(s): B3KTR2
, B4E1S7, Q6FG28, Q96CQ9
Entry historyiIntegrated into UniProtKB/Swiss-Prot: October 18, 2001
Last sequence update: January 24, 2006
Last modified: November 7, 2018
This is version 177 of the entry and version 2 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

3D-structure, Complete proteome, Direct protein sequencing, Reference proteome

Documents

  1. Human chromosome 4
    Human chromosome 4: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
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Main funding by: National Institutes of Health

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