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6nyy

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Current revision (09:22, 20 March 2024) (edit) (undo)
 
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<SX load='6nyy' size='340' side='right' viewer='molstar' caption='[[6nyy]], [[Resolution|resolution]] 3.00&Aring;' scene=''>
<SX load='6nyy' size='340' side='right' viewer='molstar' caption='[[6nyy]], [[Resolution|resolution]] 3.00&Aring;' scene=''>
== Structural highlights ==
== Structural highlights ==
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<table><tr><td colspan='2'>[[6nyy]] is a 10 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=6NYY OCA]. For a <b>guided tour on the structure components</b> use [http://proteopedia.org/fgij/fg.htm?mol=6NYY FirstGlance]. <br>
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<table><tr><td colspan='2'>[[6nyy]] is a 10 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=6NYY OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=6NYY FirstGlance]. <br>
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</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=ADP:ADENOSINE-5-DIPHOSPHATE'>ADP</scene>, <scene name='pdbligand=ANP:PHOSPHOAMINOPHOSPHONIC+ACID-ADENYLATE+ESTER'>ANP</scene>, <scene name='pdbligand=MG:MAGNESIUM+ION'>MG</scene>, <scene name='pdbligand=ZN:ZINC+ION'>ZN</scene></td></tr>
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</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">Electron Microscopy, [[Resolution|Resolution]] 3&#8491;</td></tr>
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<tr id='gene'><td class="sblockLbl"><b>[[Gene|Gene:]]</b></td><td class="sblockDat">AFG3L2 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 HUMAN])</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=ADP:ADENOSINE-5-DIPHOSPHATE'>ADP</scene>, <scene name='pdbligand=ANP:PHOSPHOAMINOPHOSPHONIC+ACID-ADENYLATE+ESTER'>ANP</scene>, <scene name='pdbligand=MG:MAGNESIUM+ION'>MG</scene>, <scene name='pdbligand=ZN:ZINC+ION'>ZN</scene></td></tr>
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<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://proteopedia.org/fgij/fg.htm?mol=6nyy FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6nyy OCA], [http://pdbe.org/6nyy PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=6nyy RCSB], [http://www.ebi.ac.uk/pdbsum/6nyy PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=6nyy ProSAT]</span></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=6nyy FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6nyy OCA], [https://pdbe.org/6nyy PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=6nyy RCSB], [https://www.ebi.ac.uk/pdbsum/6nyy PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=6nyy ProSAT]</span></td></tr>
</table>
</table>
== Disease ==
== Disease ==
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[[http://www.uniprot.org/uniprot/AFG32_HUMAN AFG32_HUMAN]] Spinocerebellar ataxia type 28;Early-onset spastic ataxia-neuropathy syndrome. The disease is caused by mutations affecting the gene represented in this entry. The disease is caused by mutations affecting the gene represented in this entry.
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[https://www.uniprot.org/uniprot/AFG32_HUMAN AFG32_HUMAN] Spinocerebellar ataxia type 28;Early-onset spastic ataxia-neuropathy syndrome. The disease is caused by mutations affecting the gene represented in this entry. The disease is caused by mutations affecting the gene represented in this entry.
== Function ==
== Function ==
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[[http://www.uniprot.org/uniprot/AFG32_HUMAN AFG32_HUMAN]] ATP-dependent protease which is essential for axonal development (By similarity).
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[https://www.uniprot.org/uniprot/AFG32_HUMAN AFG32_HUMAN] ATP-dependent protease which is essential for axonal development (By similarity).
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<div style="background-color:#fffaf0;">
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== Publication Abstract from PubMed ==
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Mitochondrial AAA+ quality-control proteases regulate diverse aspects of mitochondrial biology through specialized protein degradation, but the underlying mechanisms of these enzymes remain poorly defined. The mitochondrial AAA+ protease AFG3L2 is of particular interest, as genetic mutations localized throughout AFG3L2 are linked to diverse neurodegenerative disorders. However, a lack of structural data has limited our understanding of how mutations impact enzymatic function. Here, we used cryoelectron microscopy (cryo-EM) to determine a substrate-bound structure of the catalytic core of human AFG3L2. This structure identifies multiple specialized structural features that integrate with conserved motifs required for ATP-dependent translocation to unfold and degrade targeted proteins. Many disease-relevant mutations localize to these unique structural features of AFG3L2 and distinctly influence its activity and stability. Our results provide a molecular basis for neurological phenotypes associated with different AFG3L2 mutations and establish a structural framework to understand how different members of the AAA+ superfamily achieve specialized biological functions.
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Unique Structural Features of the Mitochondrial AAA+ Protease AFG3L2 Reveal the Molecular Basis for Activity in Health and Disease.,Puchades C, Ding B, Song A, Wiseman RL, Lander GC, Glynn SE Mol Cell. 2019 Jul 11. pii: S1097-2765(19)30472-1. doi:, 10.1016/j.molcel.2019.06.016. PMID:31327635<ref>PMID:31327635</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 6nyy" style="background-color:#fffaf0;"></div>
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== References ==
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<references/>
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__TOC__
__TOC__
</SX>
</SX>
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[[Category: Human]]
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[[Category: Homo sapiens]]
[[Category: Large Structures]]
[[Category: Large Structures]]
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[[Category: Lander, G C]]
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[[Category: Lander GC]]
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[[Category: Puchades, C]]
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[[Category: Puchades C]]
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[[Category: Aaa+]]
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[[Category: Afg3l2]]
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[[Category: Atpase]]
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[[Category: Inner membrane]]
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[[Category: M/aaa protease]]
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[[Category: Mitochondria]]
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[[Category: Neurodegeneration]]
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[[Category: Protease]]
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[[Category: Protein quality control]]
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[[Category: Translocase]]
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Current revision

human m-AAA protease AFG3L2, substrate-bound

6nyy, resolution 3.00Å

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