4aoh

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[[Image:4aoh.png|left|200px]]
 
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{{STRUCTURE_4aoh| PDB=4aoh | SCENE= }}
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==Structural snapshots and functional analysis of human angiogenin variants associated with Amyotrophic Lateral Sclerosis (ALS)==
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<StructureSection load='4aoh' size='340' side='right'caption='[[4aoh]], [[Resolution|resolution]] 1.04&Aring;' scene=''>
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== Structural highlights ==
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<table><tr><td colspan='2'>[[4aoh]] is a 1 chain structure with sequence from [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4AOH OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=4AOH FirstGlance]. <br>
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</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution|Resolution]] 1.041&#8491;</td></tr>
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<tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=TAR:D(-)-TARTARIC+ACID'>TAR</scene>, <scene name='pdbligand=TLA:L(+)-TARTARIC+ACID'>TLA</scene></td></tr>
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<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=4aoh FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4aoh OCA], [https://pdbe.org/4aoh PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=4aoh RCSB], [https://www.ebi.ac.uk/pdbsum/4aoh PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=4aoh ProSAT]</span></td></tr>
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</table>
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== Disease ==
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[https://www.uniprot.org/uniprot/ANGI_HUMAN ANGI_HUMAN] Defects in ANG are the cause of susceptibility to amyotrophic lateral sclerosis type 9 (ALS9) [MIM:[https://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>
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== Function ==
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[https://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>
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<div style="background-color:#fffaf0;">
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== Publication Abstract from PubMed ==
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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.
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===Structural snapshots and functional analysis of human angiogenin variants associated with Amyotrophic Lateral Sclerosis (ALS)===
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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>
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From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
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</div>
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<div class="pdbe-citations 4aoh" style="background-color:#fffaf0;"></div>
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==About this Structure==
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==See Also==
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[[4aoh]] 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=4AOH OCA].
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*[[Ribonuclease 3D structures|Ribonuclease 3D structures]]
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== References ==
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<references/>
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__TOC__
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</StructureSection>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Acharya, K R.]]
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[[Category: Large Structures]]
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[[Category: Ferguson, R.]]
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[[Category: Acharya KR]]
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[[Category: Subramanian, V.]]
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[[Category: Ferguson R]]
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[[Category: Thiyagarajan, N.]]
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[[Category: Subramanian V]]
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[[Category: Al]]
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[[Category: Thiyagarajan N]]
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[[Category: Amyotropic lateral sclerosis]]
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[[Category: Angiogenesis]]
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[[Category: Hydrolase]]
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[[Category: Motor neuron disease]]
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[[Category: Neovascularisation]]
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[[Category: Nuclear localisation]]
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[[Category: Nuclear localization]]
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[[Category: Ribonuclease inhibitor]]
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Current revision

Structural snapshots and functional analysis of human angiogenin variants associated with Amyotrophic Lateral Sclerosis (ALS)

PDB ID 4aoh

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