3ikl

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{{STRUCTURE_3ikl| PDB=3ikl | SCENE= }}
 
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===Crystal structure of Pol gB delta-I4.===
 
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{{ABSTRACT_PUBMED_19837034}}
 
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==Disease==
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==Crystal structure of Pol gB delta-I4.==
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[[http://www.uniprot.org/uniprot/DPOG2_HUMAN DPOG2_HUMAN]] Defects in POLG2 are the cause of progressive external ophthalmoplegia with mitochondrial DNA deletions autosomal dominant type 4 (PEOA4) [MIM:[http://omim.org/entry/610131 610131]]. Progressive external ophthalmoplegia is characterized by progressive weakness of ocular muscles and levator muscle of the upper eyelid. In a minority of cases, it is associated with skeletal myopathy, which predominantly involves axial or proximal muscles and which causes abnormal fatigability and even permanent muscle weakness. Ragged-red fibers and atrophy are found on muscle biopsy. A large proportion of chronic ophthalmoplegias are associated with other symptoms, leading to a multisystemic pattern of this disease. Additional symptoms are variable, and may include cataracts, hearing loss, sensory axonal neuropathy, ataxia, depression, hypogonadism, and parkinsonism.<ref>PMID:16685652</ref>
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<StructureSection load='3ikl' size='340' side='right'caption='[[3ikl]], [[Resolution|resolution]] 3.10&Aring;' scene=''>
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== Structural highlights ==
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==Function==
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<table><tr><td colspan='2'>[[3ikl]] is a 2 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=3IKL OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=3IKL FirstGlance]. <br>
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[[http://www.uniprot.org/uniprot/DPOG2_HUMAN DPOG2_HUMAN]] Mitochondrial polymerase processivity subunit. Stimulates the polymerase and exonuclease activities, and increases the processivity of the enzyme. Binds to ss-DNA.
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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]] 3.1&#8491;</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=3ikl FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3ikl OCA], [https://pdbe.org/3ikl PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=3ikl RCSB], [https://www.ebi.ac.uk/pdbsum/3ikl PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=3ikl ProSAT]</span></td></tr>
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==About this Structure==
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</table>
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[[3ikl]] is a 2 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=3IKL OCA].
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== Disease ==
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[https://www.uniprot.org/uniprot/DPOG2_HUMAN DPOG2_HUMAN] Defects in POLG2 are the cause of progressive external ophthalmoplegia with mitochondrial DNA deletions autosomal dominant type 4 (PEOA4) [MIM:[https://omim.org/entry/610131 610131]. Progressive external ophthalmoplegia is characterized by progressive weakness of ocular muscles and levator muscle of the upper eyelid. In a minority of cases, it is associated with skeletal myopathy, which predominantly involves axial or proximal muscles and which causes abnormal fatigability and even permanent muscle weakness. Ragged-red fibers and atrophy are found on muscle biopsy. A large proportion of chronic ophthalmoplegias are associated with other symptoms, leading to a multisystemic pattern of this disease. Additional symptoms are variable, and may include cataracts, hearing loss, sensory axonal neuropathy, ataxia, depression, hypogonadism, and parkinsonism.<ref>PMID:16685652</ref>
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== Function ==
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[https://www.uniprot.org/uniprot/DPOG2_HUMAN DPOG2_HUMAN] Mitochondrial polymerase processivity subunit. Stimulates the polymerase and exonuclease activities, and increases the processivity of the enzyme. Binds to ss-DNA.
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== Evolutionary Conservation ==
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[[Image:Consurf_key_small.gif|200px|right]]
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Check<jmol>
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<jmolCheckbox>
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<scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/ik/3ikl_consurf.spt"</scriptWhenChecked>
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<scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked>
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<text>to colour the structure by Evolutionary Conservation</text>
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</jmolCheckbox>
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</jmol>, as determined by [http://consurfdb.tau.ac.il/ ConSurfDB]. You may read the [[Conservation%2C_Evolutionary|explanation]] of the method and the full data available from [http://bental.tau.ac.il/new_ConSurfDB/main_output.php?pdb_ID=3ikl ConSurf].
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<div style="clear:both"></div>
==See Also==
==See Also==
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*[[DNA polymerase|DNA polymerase]]
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*[[DNA polymerase 3D structures|DNA polymerase 3D structures]]
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== References ==
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==Reference==
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<references/>
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<ref group="xtra">PMID:019837034</ref><references group="xtra"/><references/>
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__TOC__
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[[Category: DNA-directed DNA polymerase]]
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</StructureSection>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Kennedy, W D.]]
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[[Category: Large Structures]]
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[[Category: Lee, Y S.]]
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[[Category: Kennedy WD]]
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[[Category: Yin, Y W.]]
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[[Category: Lee YS]]
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[[Category: Transferase]]
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[[Category: Yin YW]]

Current revision

Crystal structure of Pol gB delta-I4.

PDB ID 3ikl

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