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FLASH GENE
Symbol AIMP1 contributors: mct - updated : 05-07-2017
HGNC name aminoacyl tRNA synthetase complex-interacting multifunctional protein 1
HGNC id 10648
Corresponding disease
PMLD3 Pelizaeus-Merzbacher-like disease 3
Location 4q24      Physical location : 107.236.766 - 107.270.379
Synonym name
  • endothelial monocyte-activating polypeptide
  • ARS-interacting multifunctional prtotein 1
  • Apoptosis-released Factor
  • small inducible cytokine subfamily E, member 1 (endothelial monocyte-activating)
  • multisynthetase complex p43
  • Synonym symbol(s) p43, EMAP2, EMAPII, EMAP-2, p43(ARF), SCYE1, MSCp43
    DNA
    TYPE functioning gene
    STRUCTURE 33.61 kb     7 Exon(s)
    MAPPING cloned Y linked N status confirmed
    RNA
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    7 - 2537 - 312 - 1994 7545917
    7 - 2598 - 336 - 1994 7545917
    7 - 3159 - 312 - 1994 7545917
    EXPRESSION
    Type ubiquitous
       expressed in (based on citations)
    organ(s)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Digestiveesophagus   highly
    Hearing/Equilibriumearinnercochlea highly
    Lymphoid/Immunelymph node   highly
    Nervousnerve   predominantly
    tissue
    SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
    Connectivebone  highly
    Epithelialsecretoryglandularendocrine 
    cells
    SystemCellPubmedSpeciesStageRna symbol
    Cardiovascularendothelial cell
    Endocrineislet cell (alpha,beta...)
    Nervousneuron
    cell lineage
    cell lines
    fluid/secretion
    at STAGE
    PROTEIN
    PHYSICAL PROPERTIES
    STRUCTURE
    motifs/domains
  • 72 AA N-terminal extension within the multi-ARS complex, helping the catalytic activity and cellular stability of the bound enzyme, and binds arginyl-tRNA synthetase, and N terminus region 170 aa is a binding partner of the MSC complex RARS
  • one tRNA-binding domain in the precursor form
  • central part holds the C-terminal domain of heat shock protein gp96 in endoplasmic reticulum, thereby preventing false activation of autoimmune reaction
  • N terminus of AIMP1 can bind with its C terminus to repress punctate structure formation, that it has a putative leucine zipper, that it is the region responsible for the binding of MSC subunits
  • isoforms Precursor about 20kDa after proteolytic cleavage in tumor cells. Several forms like p43(EMAPII) and p43(ARF) produced by cleavage
    HOMOLOGY
    Homologene
    FAMILY
    CATEGORY RNA associated , signaling cytokine
    SUBCELLULAR LOCALIZATION extracellular
        intracellular
    intracellular,cytoplasm,organelle,Golgi
    intracellular,cytoplasm,cytosolic
    intracellular,nucleus
    text normally retained intracellularly but can be released after proteolytic cleavage due to apoptosis and hypoxia
    basic FUNCTION
  • may function in binding tRNA in normal cells and in stimulating inflammatory response after proteolytic cleavage in tumor cells
  • mediator of the activation of endothelial cells and the activation and chemotaxis of neutrophils ans mononuclear phagocytes
  • sensitizes the tumor-associated vasculature to tumor necrosis factor
  • plays a role in accumultaion of macrophages at sites of its expression in uveal melanoma
  • may have a role in the pathophysiology of secondary injury following spinal cord injury (SCI)
  • also involved in embryonic development
  • cytokine working in the control of angiogenesis, inflammation, and wound healing
  • playing a glucagon-like role in glucose homeostasis
  • negatively regulates TGFB signaling via stabilization of SMURF2
  • having a critical role for the suppression of lupus-type autoimmune disease through the interaction with ER-resident chaperone, gp96
  • factor associated with a macromolecular aminoacyl-tRNA synthetase (ARS) complex but also plays diverse regulatory roles in various physiological processes
  • having a activity as a component of negative feedback loop of TGF-beta signaling
  • essential for neurofilaments assembly and axon maintenance and indispensable in maintaining axonal integrity
  • negative regulator of neurofilaments phosphorylation
  • may be playing q role in the pathogenesis of CMT neuropathy type 2D (CMT2D)
  • CELLULAR PROCESS cell life, proliferation/growth
    PHYSIOLOGICAL PROCESS inflammation
    PATHWAY
    metabolism
    signaling signal transduction
    a component
  • component of the multi-tRNA synthetase complex
  • INTERACTION
    DNA
    RNA tRNA binding
    small molecule
    protein
  • binds to WW domains of SMURF2 (interaction of the C-terminal region of SCYE1 directly with the Smad7-binding region of SMURF2)
  • negatively regulates TGF-beta signaling via stabilization of SMURF2
  • into the cytoplasmic compartment interacts with its cytoplasmic partner PSMA7, a component of the proteasome degradation pathway
  • cofactor of the macromolecular ARS (aminoacyl-tRNA synthetase) complex
  • interacting directly with neurofilament-light subunit (Zhu 2009)
  • can form a molecular complex with HSP90B1, regulate the ER retention of HSP90B1 through KDELR1, and suppress its cell surface expression
  • binds and helps the catalytic reaction of arginyl-tRNA synthetase
  • importance of interactions between the N-terminal domains of RARS and AIMP1 for the catalytic and noncatalytic activities of RARS and for the assembly of the higher-order multisynthetase complex (MSC) protein complex
  • cell & other
    REGULATION
    induced by apoptosis
    inhibited by PGE2
    repressed by RARS over-expression associated with a reduced AIMP1 secretion
    Other could be phosphorylated by activation of JNK through TLR4-MYD88 pathway
    ASSOCIATED DISORDERS
    corresponding disease(s) PMLD3
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    constitutional     --low  
    resulting in the dramatic reduction of plasma gucagon levels, and inducing pancreatic secretion of glucagon for blood glucose homeostasis
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS
    Mice lacking MSC p43 exhibited axon degeneration in motor neurons, defective neuromuscular junctions, muscular atrophy, and motor dysfunction