2vgf: Difference between revisions

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[[Image:2vgf.png|left|200px]]


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==HUMAN ERYTHROCYTE PYRUVATE KINASE: T384M mutant==
The line below this paragraph, containing "STRUCTURE_2vgf", creates the "Structure Box" on the page.
<StructureSection load='2vgf' size='340' side='right'caption='[[2vgf]], [[Resolution|resolution]] 2.75&Aring;' scene=''>
You may change the PDB parameter (which sets the PDB file loaded into the applet)
== Structural highlights ==
or the SCENE parameter (which sets the initial scene displayed when the page is loaded),
<table><tr><td colspan='2'>[[2vgf]] is a 4 chain structure with sequence from [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. This structure supersedes the now removed PDB entry [http://oca.weizmann.ac.il/oca-bin/send-pdb?obs=1&id=1liw 1liw]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2VGF OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=2VGF FirstGlance]. <br>
or leave the SCENE parameter empty for the default display.
</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution|Resolution]] 2.75&#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=FBP:BETA-FRUCTOSE-1,6-DIPHOSPHATE'>FBP</scene>, <scene name='pdbligand=K:POTASSIUM+ION'>K</scene>, <scene name='pdbligand=MN:MANGANESE+(II)+ION'>MN</scene>, <scene name='pdbligand=PGA:2-PHOSPHOGLYCOLIC+ACID'>PGA</scene></td></tr>
{{STRUCTURE_2vgf|  PDB=2vgf  |  SCENE=  }}
<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=2vgf FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2vgf OCA], [https://pdbe.org/2vgf PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2vgf RCSB], [https://www.ebi.ac.uk/pdbsum/2vgf PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2vgf ProSAT]</span></td></tr>
 
</table>
===HUMAN ERYTHROCYTE PYRUVATE KINASE: T384M MUTANT===
== Disease ==
 
[https://www.uniprot.org/uniprot/KPYR_HUMAN KPYR_HUMAN] Defects in PKLR are the cause of pyruvate kinase hyperactivity (PKHYP) [MIM:[https://omim.org/entry/102900 102900]; also known as high red cell ATP syndrome. This autosomal dominant phenotype is characterized by increase of red blood cell ATP.<ref>PMID:9090535</ref>  Defects in PKLR are the cause of pyruvate kinase deficiency of red cells (PKRD) [MIM:[https://omim.org/entry/266200 266200]. A frequent cause of hereditary non-spherocytic hemolytic anemia. Clinically, pyruvate kinase-deficient patients suffer from a highly variable degree of chronic hemolysis, ranging from severe neonatal jaundice and fatal anemia at birth, severe transfusion-dependent chronic hemolysis, moderate hemolysis with exacerbation during infection, to a fully compensated hemolysis without apparent anemia.
 
== Function ==
<!--
[https://www.uniprot.org/uniprot/KPYR_HUMAN KPYR_HUMAN] Plays a key role in glycolysis (By similarity).
The line below this paragraph, {{ABSTRACT_PUBMED_11960989}}, adds the Publication Abstract to the page
== Evolutionary Conservation ==
(as it appears on PubMed at http://www.pubmed.gov), where 11960989 is the PubMed ID number.
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Check<jmol>
{{ABSTRACT_PUBMED_11960989}}
  <jmolCheckbox>
 
    <scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/vg/2vgf_consurf.spt"</scriptWhenChecked>
==About this Structure==
    <scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked>
2VGF is a [[Single protein]] structure of sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. This structure supersedes the now removed PDB entry [http://oca.weizmann.ac.il/oca-bin/send-pdb?obs=1&id=1liw 1liw]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2VGF OCA].  
    <text>to colour the structure by Evolutionary Conservation</text>
 
  </jmolCheckbox>
==Reference==
</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=2vgf ConSurf].
Structure and function of human erythrocyte pyruvate kinase. Molecular basis of nonspherocytic hemolytic anemia., Valentini G, Chiarelli LR, Fortin R, Dolzan M, Galizzi A, Abraham DJ, Wang C, Bianchi P, Zanella A, Mattevi A, J Biol Chem. 2002 Jun 28;277(26):23807-14. Epub 2002 Apr 17. PMID:[http://www.ncbi.nlm.nih.gov/pubmed/11960989 11960989]
<div style="clear:both"></div>
== References ==
<references/>
__TOC__
</StructureSection>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
[[Category: Pyruvate kinase]]
[[Category: Large Structures]]
[[Category: Single protein]]
[[Category: Abraham DJ]]
[[Category: Abraham, D J.]]
[[Category: Bianchi P]]
[[Category: Bianchi, P.]]
[[Category: Chiarelli LR]]
[[Category: Chiarelli, L.]]
[[Category: Dolzan M]]
[[Category: Dolzan, M.]]
[[Category: Fortin R]]
[[Category: Fortin, R.]]
[[Category: Galizzi A]]
[[Category: Galizzi, A.]]
[[Category: Mattevi A]]
[[Category: Mattevi, A.]]
[[Category: Valentini G]]
[[Category: Valentini, G.]]
[[Category: Wang C]]
[[Category: Wang, C.]]
[[Category: Zanella A]]
[[Category: Zanella, A.]]
[[Category: Alternative splicing]]
[[Category: Disease mutation]]
[[Category: Glycolysis]]
[[Category: Kinase]]
[[Category: Magnesium]]
[[Category: Metal-binding]]
[[Category: Phosphorylation]]
[[Category: Polymorphism]]
[[Category: Pyruvate]]
[[Category: Pyruvate kinase in the active r-state]]
[[Category: Transferase]]
 
''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Sun Jul 27 15:19:01 2008''

Latest revision as of 10:07, 1 May 2024

HUMAN ERYTHROCYTE PYRUVATE KINASE: T384M mutantHUMAN ERYTHROCYTE PYRUVATE KINASE: T384M mutant

Structural highlights

2vgf is a 4 chain structure with sequence from Homo sapiens. This structure supersedes the now removed PDB entry 1liw. Full crystallographic information is available from OCA. For a guided tour on the structure components use FirstGlance.
Method:X-ray diffraction, Resolution 2.75Å
Ligands:, , ,
Resources:FirstGlance, OCA, PDBe, RCSB, PDBsum, ProSAT

Disease

KPYR_HUMAN Defects in PKLR are the cause of pyruvate kinase hyperactivity (PKHYP) [MIM:102900; also known as high red cell ATP syndrome. This autosomal dominant phenotype is characterized by increase of red blood cell ATP.[1] Defects in PKLR are the cause of pyruvate kinase deficiency of red cells (PKRD) [MIM:266200. A frequent cause of hereditary non-spherocytic hemolytic anemia. Clinically, pyruvate kinase-deficient patients suffer from a highly variable degree of chronic hemolysis, ranging from severe neonatal jaundice and fatal anemia at birth, severe transfusion-dependent chronic hemolysis, moderate hemolysis with exacerbation during infection, to a fully compensated hemolysis without apparent anemia.

Function

KPYR_HUMAN Plays a key role in glycolysis (By similarity).

Evolutionary Conservation

Check, as determined by ConSurfDB. You may read the explanation of the method and the full data available from ConSurf.

References

  1. ↑ Beutler E, Westwood B, van Zwieten R, Roos D. G-->T transition at cDNA nt 110 (K37Q) in the PKLR (pyruvate kinase) gene is the molecular basis of a case of hereditary increase of red blood cell ATP. Hum Mutat. 1997;9(3):282-5. PMID:9090535 doi:<282::AID-HUMU13>3.0.CO;2-Z 10.1002/(SICI)1098-1004(1997)9:3<282::AID-HUMU13>3.0.CO;2-Z

2vgf, resolution 2.75Å

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