4r3z
From Proteopedia
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| - | ''' | + | ==Translation complex== |
| - | + | <StructureSection load='4r3z' size='340' side='right' caption='[[4r3z]], [[Resolution|resolution]] 4.03Å' scene=''> | |
| - | + | == Structural highlights == | |
| - | + | <table><tr><td colspan='2'>[[4r3z]] is a 3 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4R3Z OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=4R3Z FirstGlance]. <br> | |
| - | + | </td></tr><tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Arginine--tRNA_ligase Arginine--tRNA ligase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=6.1.1.19 6.1.1.19] </span></td></tr> | |
| - | + | <tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=4r3z FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4r3z OCA], [http://www.rcsb.org/pdb/explore.do?structureId=4r3z RCSB], [http://www.ebi.ac.uk/pdbsum/4r3z PDBsum]</span></td></tr> | |
| - | + | </table> | |
| + | == Disease == | ||
| + | [[http://www.uniprot.org/uniprot/AIMP1_HUMAN AIMP1_HUMAN]] Defects in AIMP1 are the cause of leukodystrophy hypomyelinating type 3 (HLD3) [MIM:[http://omim.org/entry/260600 260600]]. A 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.<ref>PMID:21092922</ref> [[http://www.uniprot.org/uniprot/SYQ_HUMAN SYQ_HUMAN]] The disease is caused by mutations affecting the gene represented in this entry. | ||
| + | == Function == | ||
| + | [[http://www.uniprot.org/uniprot/AIMP1_HUMAN AIMP1_HUMAN]] Non-catalytic component of the multisynthase complex. Stimulates the catalytic activity of cytoplasmic arginyl-tRNA synthase. Binds tRNA. Possesses inflammatory cytokine activity. Negatively regulates TGF-beta signaling through stabilization of SMURF2 by binding to SMURF2 and inhibiting its SMAD7-mediated degradation. Involved in glucose homeostasis through induction of glucagon secretion at low glucose levels. Promotes dermal fibroblast proliferation and wound repair. Regulates KDELR1-mediated retention of HSP90B1/gp96 in the endoplasmic reticulum. Plays a role in angiogenesis by inducing endothelial cell migration at low concentrations and endothelian cell apoptosis at high concentrations. Induces maturation of dendritic cells and monocyte cell adhesion. Modulates endothelial cell responses by degrading HIF-1A through interaction with PSMA7.<ref>PMID:10358004</ref> <ref>PMID:11306575</ref> <ref>PMID:12237313</ref> <ref>PMID:11818442</ref> <ref>PMID:19362550</ref> <ref>PMID:11157763</ref> [[http://www.uniprot.org/uniprot/SYQ_HUMAN SYQ_HUMAN]] Plays a critical role in brain development.<ref>PMID:24656866</ref> [[http://www.uniprot.org/uniprot/SYRC_HUMAN SYRC_HUMAN]] Forms part of a macromolecular complex that catalyzes the attachment of specific amino acids to cognate tRNAs during protein synthesis. Modulates the secretion of AIMP1 and may be involved in generation of the inflammatory cytokine EMAP2 from AIMP1.<ref>PMID:17443684</ref> | ||
| + | == References == | ||
| + | <references/> | ||
| + | __TOC__ | ||
| + | </StructureSection> | ||
| + | [[Category: Arginine--tRNA ligase]] | ||
| + | [[Category: Cho, Y.]] | ||
| + | [[Category: Fu, Y.]] | ||
| + | [[Category: Kim, Y.]] | ||
| + | [[Category: Amino-acyl trna synthetase complex]] | ||
| + | [[Category: Ligation amino acid to trna]] | ||
| + | [[Category: Multi-synthetase complex]] | ||
| + | [[Category: Protein binding-ligase complex]] | ||
Revision as of 07:54, 8 October 2014
Translation complex
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