4ahn: Difference between revisions
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==R121H - Angiogenin mutants and amyotrophic lateral sclerosis - a biochemical and biological analysis== | ==R121H - Angiogenin mutants and amyotrophic lateral sclerosis - a biochemical and biological analysis== | ||
<StructureSection load='4ahn' size='340' side='right' caption='[[4ahn]], [[Resolution|resolution]] 2.98Å' scene=''> | <StructureSection load='4ahn' size='340' side='right' caption='[[4ahn]], [[Resolution|resolution]] 2.98Å' scene=''> | ||
== Structural highlights == | == Structural highlights == | ||
<table><tr><td colspan='2'>[[4ahn]] is a 1 chain structure with sequence from [http://en.wikipedia.org/wiki/ | <table><tr><td colspan='2'>[[4ahn]] is a 1 chain structure with sequence from [http://en.wikipedia.org/wiki/Human Human]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4AHN OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=4AHN FirstGlance]. <br> | ||
</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=CL:CHLORIDE+ION'>CL</scene></td></tr> | </td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=CL:CHLORIDE+ION'>CL</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]], [[4ahj|4ahj]], [[4ahk|4ahk]], [[4ahe|4ahe]], [[4ahm|4ahm]], [[4ahf|4ahf]], [[4ahd|4ahd]], [[4ahg|4ahg]], [[4ahi|4ahi]], [[4ahh|4ahh]], [[4ahl|4ahl]]</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]], [[4ahj|4ahj]], [[4ahk|4ahk]], [[4ahe|4ahe]], [[4ahm|4ahm]], [[4ahf|4ahf]], [[4ahd|4ahd]], [[4ahg|4ahg]], [[4ahi|4ahi]], [[4ahh|4ahh]], [[4ahl|4ahl]]</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=4ahn FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4ahn OCA], [http://www.rcsb.org/pdb/explore.do?structureId=4ahn RCSB], [http://www.ebi.ac.uk/pdbsum/4ahn PDBsum]</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=4ahn FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4ahn OCA], [http://pdbe.org/4ahn PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=4ahn RCSB], [http://www.ebi.ac.uk/pdbsum/4ahn PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=4ahn ProSAT]</span></td></tr> | ||
</table> | </table> | ||
== Disease == | == Disease == | ||
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.<br> | From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.<br> | ||
</div> | </div> | ||
<div class="pdbe-citations 4ahn" style="background-color:#fffaf0;"></div> | |||
==See Also== | |||
*[[Ribonuclease|Ribonuclease]] | |||
== References == | == References == | ||
<references/> | <references/> | ||
__TOC__ | __TOC__ | ||
</StructureSection> | </StructureSection> | ||
[[Category: | [[Category: Human]] | ||
[[Category: Acharya, K R]] | [[Category: Acharya, K R]] | ||
[[Category: Ferguson, R]] | [[Category: Ferguson, R]] | ||
Revision as of 14:48, 5 August 2016
R121H - Angiogenin mutants and amyotrophic lateral sclerosis - a biochemical and biological analysisR121H - Angiogenin mutants and amyotrophic lateral sclerosis - a biochemical and biological analysis
Structural highlights
Disease[ANGI_HUMAN] Defects in ANG are the cause of susceptibility to amyotrophic lateral sclerosis type 9 (ALS9) [MIM: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.[1] [2] [3] [4] [5] [6] Function[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.[7] [8] Publication Abstract from PubMedMutations 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[9] From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine. See AlsoReferences
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