8d42 - Revision history

OCA at 05:53, 5 July 2023

2023-07-05T05:53:16Z

←Older revision		Revision as of 05:53, 5 July 2023
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	== Structural highlights ==		== Structural highlights ==
	<table><tr><td colspan='2'>[[8d42]] is a 5 chain structure with sequence from [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens] and [https://en.wikipedia.org/wiki/Synthetic_construct Synthetic construct]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=8D42 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=8D42 FirstGlance]. <br>		<table><tr><td colspan='2'>[[8d42]] is a 5 chain structure with sequence from [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens] and [https://en.wikipedia.org/wiki/Synthetic_construct Synthetic construct]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=8D42 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=8D42 FirstGlance]. <br>
-	</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=CA:CALCIUM+ION'>CA</scene>, <scene name='pdbligand=DCP:2-DEOXYCYTIDINE-5-TRIPHOSPHATE'>DCP</scene></td></tr>	+	</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models\|Method:]]</b></td><td class="sblockDat" id="methodDat">Electron Microscopy, [[Resolution\|Resolution]] 2.91Å</td></tr>
		+	<tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=CA:CALCIUM+ION'>CA</scene>, <scene name='pdbligand=DCP:2-DEOXYCYTIDINE-5-TRIPHOSPHATE'>DCP</scene></td></tr>
	<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=8d42 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=8d42 OCA], [https://pdbe.org/8d42 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=8d42 RCSB], [https://www.ebi.ac.uk/pdbsum/8d42 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=8d42 ProSAT]</span></td></tr>		<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=8d42 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=8d42 OCA], [https://pdbe.org/8d42 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=8d42 RCSB], [https://www.ebi.ac.uk/pdbsum/8d42 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=8d42 ProSAT]</span></td></tr>
	</table>		</table>

OCA at 04:03, 25 May 2023

2023-05-25T04:03:41Z

←Older revision		Revision as of 04:03, 25 May 2023
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-	'''Unreleased structure'''

-	~~The entry~~ 8d42 is ~~ON HOLD~~ ~~until Paper Publication~~	+	==Human mitochondrial DNA polymerase gamma ternary complex with GT basepair in editing conformer (composite)==
-		+	<StructureSection load='8d42' size='340' side='right'caption='[[8d42]], [[Resolution\|resolution]] 2.91Å' scene=''>
-	~~Authors~~:	+	== Structural highlights ==
-		+	<table><tr><td colspan='2'>[[8d42]] is a 5 chain structure with sequence from [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens] and [https://en.wikipedia.org/wiki/Synthetic_construct Synthetic construct]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=8D42 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=8D42 FirstGlance]. <br>
-	~~Description~~:	+	</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=CA:CALCIUM+ION'>CA</scene>, <scene name='pdbligand=DCP:2-DEOXYCYTIDINE-5-TRIPHOSPHATE'>DCP</scene></td></tr>
-	[[Category: ~~Unreleased~~ Structures]]	+	<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=8d42 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=8d42 OCA], [https://pdbe.org/8d42 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=8d42 RCSB], [https://www.ebi.ac.uk/pdbsum/8d42 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=8d42 ProSAT]</span></td></tr>
		+	</table>
		+	== Disease ==
		+	[https://www.uniprot.org/uniprot/DPOG1_HUMAN DPOG1_HUMAN] Defects in POLG are the cause of progressive external ophthalmoplegia with mitochondrial DNA deletions autosomal dominant type 1 (PEOA1) [MIM:[https://omim.org/entry/157640 157640]. 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:12210792</ref> <ref>PMID:11897778</ref> <ref>PMID:15534189</ref> <ref>PMID:15351195</ref> <ref>PMID:17420318</ref> <ref>PMID:18575922</ref> Defects in POLG are a cause of progressive external ophthalmoplegia with mitochondrial DNA deletions autosomal recessive (PEOB) [MIM:[https://omim.org/entry/258450 258450]. PEOB is a severe form of progressive external ophthalmoplegia. It is clinically more heterogeneous than the autosomal dominant forms. Can be more severe.<ref>PMID:15351195</ref> <ref>PMID:11431686</ref> <ref>PMID:12975295</ref> <ref>PMID:12872260</ref> <ref>PMID:14635118</ref> <ref>PMID:12707443</ref> <ref>PMID:12565911</ref> <ref>PMID:15349879</ref> <ref>PMID:15477547</ref> <ref>PMID:15917273</ref> <ref>PMID:16634032</ref> <ref>PMID:16401742</ref> <ref>PMID:16621917</ref> <ref>PMID:16639411</ref> Defects in POLG are a cause of sensory ataxic neuropathy dysarthria and ophthalmoparesis (SANDO) [MIM:[https://omim.org/entry/607459 607459]. SANDO is a systemic disorder resulting from mitochondrial dysfunction associated with mitochondrial depletion in skeletal muscle and peripheral nerve tissue. The clinical triad of symptoms consists of sensory ataxic neuropathy, dysarthria, and ophthalmoparesis. However, the phenotype varies widely, even within the same family, and can also include myopathy, seizures, and hearing loss. An atypical form of the disease is characterized by headaches and/or seizures manifesting in childhood or adolescence, followed by development of cerebellar and sensory ataxia, dysarthria, progressive external ophthalmoplegia, and myoclonus in early adulthood.<ref>PMID:12565911</ref> <ref>PMID:15477547</ref> <ref>PMID:15917273</ref> <ref>PMID:16621917</ref> <ref>PMID:16639411</ref> <ref>PMID:14745080</ref> <ref>PMID:16080118</ref> <ref>PMID:15824347</ref> <ref>PMID:16919951</ref> Defects in POLG are the cause of mitochondrial DNA depletion syndrome type 4A (MTDPS4A) [MIM:[https://omim.org/entry/203700 203700]; also called Alpers diffuse degeneration of cerebral gray matter with hepatic cirrhosis. An autosomal recessive hepatocerebral syndrome. The typical course of the disease includes severe developmental delay, intractable seizures, liver failure, and death in childhood. Refractory seizures, cortical blindness, progressive liver dysfunction, and acute liver failure after exposure to valproic acid are considered diagnostic features. The neuropathological hallmarks are neuronal loss, spongiform degeneration, and astrocytosis of the visual cortex. Liver biopsy results show steatosis, often progressing to cirrhosis.<ref>PMID:16621917</ref> <ref>PMID:16639411</ref> <ref>PMID:15122711</ref> <ref>PMID:15929042</ref> <ref>PMID:15689359</ref> <ref>PMID:18828154</ref> Defects in POLG are the cause of mitochondrial DNA depletion syndrome type 4B (MTDPS4B) [MIM:[https://omim.org/entry/613662 613662]; also known as mitochondrial DNA depletion syndrome 4B MNGIE type or mitochondrial neurogastrointestinal encephalopathy syndrome POLG-related. An autosomal recessive progressive multisystem disorder clinically characterized by chronic gastrointestinal dysmotility and pseudo-obstruction, cachexia, progressive external ophthalmoplegia, axonal sensory ataxic neuropathy, and muscle weakness. Defects in POLG are a cause of Leigh syndrome (LS) [MIM:[https://omim.org/entry/256000 256000]. LS is a severe neurological disorder characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions.<ref>PMID:18828154</ref>
		+	== Function ==
		+	[https://www.uniprot.org/uniprot/DPOG1_HUMAN DPOG1_HUMAN] Involved in the replication of mitochondrial DNA. Associates with mitochondrial DNA.
		+	== References ==
		+	<references/>
		+	__TOC__
		+	</StructureSection>
		+	[[Category: Homo sapiens]]
		+	[[Category: Large Structures]]
		+	[[Category: Synthetic construct]]
		+	[[Category: Park J]]
		+	[[Category: Yin YW]]

OCA at 15:09, 6 July 2022

2022-07-06T15:09:52Z

←Older revision		Revision as of 15:09, 6 July 2022
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	'''Unreleased structure'''		'''Unreleased structure'''

-	The entry 8d42 is ON HOLD	+	The entry 8d42 is ON HOLD until Paper Publication

	Authors:		Authors:

OCA: Protected "8d42" [edit=sysop:move=sysop]

2022-06-08T04:36:18Z

Protected "8d42" [edit=sysop:move=sysop]

←Older revision

Revision as of 04:36, 8 June 2022

OCA: New page: '''Unreleased structure''' The entry 8d42 is ON HOLD Authors: Description: Category: Unreleased Structures

2022-06-08T04:36:17Z

New page: '''Unreleased structure''' The entry 8d42 is ON HOLD Authors: Description: Category: Unreleased Structures

New page

'''Unreleased structure'''

The entry 8d42 is ON HOLD

Authors:

Description:
[[Category: Unreleased Structures]]