4ahh
From Proteopedia
(Difference between revisions)
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| - | + | ==R31K - Angiogenin mutants and amyotrophic lateral sclerosis - a biochemical and biological analysis== | |
| - | + | <StructureSection load='4ahh' size='340' side='right' caption='[[4ahh]], [[Resolution|resolution]] 2.50Å' scene=''> | |
| - | + | == Structural highlights == | |
| + | <table><tr><td colspan='2'>[[4ahh]] is a 1 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4AHH OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=4AHH FirstGlance]. <br> | ||
| + | </td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=TLA:L(+)-TARTARIC+ACID'>TLA</scene></td></tr> | ||
| + | <tr id='related'><td class="sblockLbl"><b>[[Related_structure|Related:]]</b></td><td class="sblockDat">[[1hby|1hby]], [[1h53|1h53]], [[2ang|2ang]], [[1un3|1un3]], [[1a4y|1a4y]], [[1b1j|1b1j]], [[1un4|1un4]], [[1un5|1un5]], [[1h0d|1h0d]], [[1b1e|1b1e]], [[1k59|1k59]], [[1k5b|1k5b]], [[1k58|1k58]], [[1ang|1ang]], [[1k5a|1k5a]], [[1awz|1awz]], [[1b1i|1b1i]], [[1h52|1h52]], [[4ahf|4ahf]], [[4ahe|4ahe]], [[4ahd|4ahd]], [[4ahi|4ahi]], [[4ahj|4ahj]], [[4ahk|4ahk]], [[4ahg|4ahg]], [[4ahl|4ahl]], [[4ahm|4ahm]]</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=4ahh FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4ahh OCA], [http://www.rcsb.org/pdb/explore.do?structureId=4ahh RCSB], [http://www.ebi.ac.uk/pdbsum/4ahh PDBsum]</span></td></tr> | ||
| + | </table> | ||
| + | == Disease == | ||
| + | [[http://www.uniprot.org/uniprot/ANGI_HUMAN ANGI_HUMAN]] Defects in ANG are the cause of susceptibility to amyotrophic lateral sclerosis type 9 (ALS9) [MIM:[http://omim.org/entry/611895 611895]]. ALS is a degenerative disorder of motor neurons in the cortex, brain stem and spinal cord. ALS is characterized by muscular weakness and atrophy.<ref>PMID:17886298</ref> <ref>PMID:15557516</ref> <ref>PMID:16501576</ref> <ref>PMID:17900154</ref> <ref>PMID:18087731</ref> <ref>PMID:17703939</ref> | ||
| + | == Function == | ||
| + | [[http://www.uniprot.org/uniprot/ANGI_HUMAN ANGI_HUMAN]] May function as a tRNA-specific ribonuclease that abolishes protein synthesis by specifically hydrolyzing cellular tRNAs. Binds to actin on the surface of endothelial cells; once bound, angiogenin is endocytosed and translocated to the nucleus. Angiogenin induces vascularization of normal and malignant tissues. Angiogenic activity is regulated by interaction with RNH1 in vivo.<ref>PMID:1400510</ref> <ref>PMID:19354288</ref> | ||
| + | <div style="background-color:#fffaf0;"> | ||
| + | == Publication Abstract from PubMed == | ||
| + | Mutations in angiogenin (ANG), a member of the ribonuclease A superfamily, are associated with amyotrophic lateral sclerosis (ALS; sporadic and familial) and Parkinson's disease. We have previously shown that ANG is expressed in neurons during neuro-ectodermal differentiation, and that it has both neurotrophic and neuroprotective functions. Here we report the atomic resolution structure of native ANG and 11 ANG-ALS variants. We correlate the structural changes to the effects on neuronal survival and the ability to induce stress granules in neuronal cell lines. ANG-ALS variants that affect the structure of the catalytic site and either decrease or increase the RNase activity affect neuronal survival. Neuronal cell lines expressing the ANG-ALS variants also lack the ability to form stress granules. Our structure-function studies on these ANG-ALS variants are the first to provide insights into the cellular and molecular mechanisms underlying their role in ALS. | ||
| - | + | Structural and molecular insights into the mechanism of action of human angiogenin-ALS variants in neurons.,Thiyagarajan N, Ferguson R, Subramanian V, Acharya KR Nat Commun. 2012 Oct 9;3:1121. doi: 10.1038/ncomms2126. PMID:23047679<ref>PMID:23047679</ref> | |
| - | + | ||
| - | + | From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.<br> | |
| - | + | </div> | |
| - | + | == References == | |
| - | == | + | <references/> |
| - | + | __TOC__ | |
| - | + | </StructureSection> | |
| - | + | ||
| - | <references | + | |
[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
| - | [[Category: Acharya, K R | + | [[Category: Acharya, K R]] |
| - | [[Category: Ferguson, R | + | [[Category: Ferguson, R]] |
| - | [[Category: Pham, T | + | [[Category: Pham, T]] |
| - | [[Category: Saha, S | + | [[Category: Saha, S]] |
| - | [[Category: Subramanian, V | + | [[Category: Subramanian, V]] |
| - | [[Category: Thiyagarajan, N | + | [[Category: Thiyagarajan, N]] |
[[Category: Amyotrophic]] | [[Category: Amyotrophic]] | ||
[[Category: Hydrolase]] | [[Category: Hydrolase]] | ||
[[Category: Lateral sclerosis]] | [[Category: Lateral sclerosis]] | ||
[[Category: Neovascularisation]] | [[Category: Neovascularisation]] | ||
Revision as of 10:07, 21 December 2014
R31K - Angiogenin mutants and amyotrophic lateral sclerosis - a biochemical and biological analysis
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