2vgf

From Proteopedia

(Difference between revisions)
Jump to: navigation, search
Current revision (07:07, 1 May 2024) (edit) (undo)
 
(7 intermediate revisions not shown.)
Line 1: Line 1:
-
{{STRUCTURE_2vgf| PDB=2vgf | SCENE= }}
 
-
===HUMAN ERYTHROCYTE PYRUVATE KINASE: T384M MUTANT===
 
-
{{ABSTRACT_PUBMED_11960989}}
 
-
==Disease==
+
==HUMAN ERYTHROCYTE PYRUVATE KINASE: T384M mutant==
-
[[http://www.uniprot.org/uniprot/KPYR_HUMAN KPYR_HUMAN]] Defects in PKLR are the cause of pyruvate kinase hyperactivity (PKHYP) [MIM:[http://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:[http://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.
+
<StructureSection load='2vgf' size='340' side='right'caption='[[2vgf]], [[Resolution|resolution]] 2.75&Aring;' scene=''>
-
 
+
== Structural highlights ==
-
==Function==
+
<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>
-
[[http://www.uniprot.org/uniprot/KPYR_HUMAN KPYR_HUMAN]] Plays a key role in glycolysis (By similarity).
+
</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>
-
 
+
<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>
-
==About this Structure==
+
<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>
-
[[2vgf]] is a 4 chain structure with 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].
+
</table>
-
 
+
== Disease ==
-
==See Also==
+
[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.
-
*[[Pyruvate Kinase|Pyruvate Kinase]]
+
== Function ==
-
 
+
[https://www.uniprot.org/uniprot/KPYR_HUMAN KPYR_HUMAN] Plays a key role in glycolysis (By similarity).
-
==Reference==
+
== Evolutionary Conservation ==
-
<ref group="xtra">PMID:011960989</ref><references group="xtra"/><references/>
+
[[Image:Consurf_key_small.gif|200px|right]]
 +
Check<jmol>
 +
<jmolCheckbox>
 +
<scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/vg/2vgf_consurf.spt"</scriptWhenChecked>
 +
<scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked>
 +
<text>to colour the structure by Evolutionary Conservation</text>
 +
</jmolCheckbox>
 +
</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].
 +
<div style="clear:both"></div>
 +
== References ==
 +
<references/>
 +
__TOC__
 +
</StructureSection>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
-
[[Category: Pyruvate kinase]]
+
[[Category: Large Structures]]
-
[[Category: Abraham, D J.]]
+
[[Category: Abraham DJ]]
-
[[Category: Bianchi, P.]]
+
[[Category: Bianchi P]]
-
[[Category: Chiarelli, L R.]]
+
[[Category: Chiarelli LR]]
-
[[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: Glycolysis]]
+
-
[[Category: Metal-binding]]
+
-
[[Category: Phosphorylation]]
+
-
[[Category: R-state]]
+
-
[[Category: Transferase]]
+

Current revision

HUMAN ERYTHROCYTE PYRUVATE KINASE: T384M mutant

PDB ID 2vgf

Drag the structure with the mouse to rotate

Proteopedia Page Contributors and Editors (what is this?)

OCA

Personal tools