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1ivy

From Proteopedia

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{{Seed}}
 
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[[Image:1ivy.png|left|200px]]
 
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<!--
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==PHYSIOLOGICAL DIMER HPP PRECURSOR==
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The line below this paragraph, containing "STRUCTURE_1ivy", creates the "Structure Box" on the page.
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<StructureSection load='1ivy' size='340' side='right'caption='[[1ivy]], [[Resolution|resolution]] 2.20&Aring;' scene=''>
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You may change the PDB parameter (which sets the PDB file loaded into the applet)
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== Structural highlights ==
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or the SCENE parameter (which sets the initial scene displayed when the page is loaded),
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<table><tr><td colspan='2'>[[1ivy]] 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=1IVY OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=1IVY FirstGlance]. <br>
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or leave the SCENE parameter empty for the default display.
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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]] 2.2&#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=NAG:N-ACETYL-D-GLUCOSAMINE'>NAG</scene>, <scene name='pdbligand=NDG:2-(ACETYLAMINO)-2-DEOXY-A-D-GLUCOPYRANOSE'>NDG</scene></td></tr>
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{{STRUCTURE_1ivy| PDB=1ivy | SCENE= }}
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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=1ivy FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1ivy OCA], [https://pdbe.org/1ivy PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=1ivy RCSB], [https://www.ebi.ac.uk/pdbsum/1ivy PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=1ivy ProSAT]</span></td></tr>
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</table>
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== Disease ==
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[https://www.uniprot.org/uniprot/PPGB_HUMAN PPGB_HUMAN] Defects in CTSA are the cause of galactosialidosis (GSL) [MIM:[https://omim.org/entry/256540 256540]. A lysosomal storage disease associated with a combined deficiency of beta-galactosidase and neuraminidase, secondary to a defect in cathepsin A. All patients have clinical manifestations typical of a lysosomal disorder, such as coarse facies, cherry red spots, vertebral changes, foam cells in the bone marrow, and vacuolated lymphocytes. Three phenotypic subtypes are recognized. The early infantile form is associated with fetal hydrops, edema, ascites, visceromegaly, skeletal dysplasia, and early death. The late infantile type is characterized by hepatosplenomegaly, growth retardation, cardiac involvement, and a normal or mildly affected mental state. The juvenile/adult form is characterized by myoclonus, ataxia, angiokeratoma, mental retardation, neurologic deterioration, absence of visceromegaly, and long survival.<ref>PMID:1756715</ref> <ref>PMID:8514852</ref> <ref>PMID:8968752</ref> <ref>PMID:10944848</ref>
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== Function ==
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[https://www.uniprot.org/uniprot/PPGB_HUMAN PPGB_HUMAN] Protective protein appears to be essential for both the activity of beta-galactosidase and neuraminidase, it associates with these enzymes and exerts a protective function necessary for their stability and activity. This protein is also a carboxypeptidase and can deamidate tachykinins.<ref>PMID:1907282</ref>
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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/iv/1ivy_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=1ivy ConSurf].
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<div style="clear:both"></div>
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===PHYSIOLOGICAL DIMER HPP PRECURSOR===
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==See Also==
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*[[Carboxypeptidase 3D structures|Carboxypeptidase 3D structures]]
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*[[Cathepsin 3D structures|Cathepsin 3D structures]]
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== References ==
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The line below this paragraph, {{ABSTRACT_PUBMED_8591035}}, adds the Publication Abstract to the page
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<references/>
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(as it appears on PubMed at http://www.pubmed.gov), where 8591035 is the PubMed ID number.
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__TOC__
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</StructureSection>
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{{ABSTRACT_PUBMED_8591035}}
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==About this Structure==
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1IVY is a [[Single protein]] structure of 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=1IVY OCA].
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==Reference==
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Three-dimensional structure of the human 'protective protein': structure of the precursor form suggests a complex activation mechanism., Rudenko G, Bonten E, d'Azzo A, Hol WG, Structure. 1995 Nov 15;3(11):1249-59. PMID:[http://www.ncbi.nlm.nih.gov/pubmed/8591035 8591035]
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[[Category: Carboxypeptidase C]]
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[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Single protein]]
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[[Category: Large Structures]]
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[[Category: Azzo, A D.]]
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[[Category: Bonten E]]
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[[Category: Bonten, E.]]
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[[Category: D'Azzo A]]
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[[Category: Hol, W G.J.]]
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[[Category: Hol WGJ]]
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[[Category: Rudenko, G.]]
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[[Category: Rudenko G]]
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[[Category: Carboxypeptidase]]
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[[Category: Glycoprotein]]
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[[Category: Protective protein]]
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[[Category: Serine carboxypeptidase]]
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[[Category: Zymogen]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Tue Jul 1 14:06:02 2008''
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Current revision

PHYSIOLOGICAL DIMER HPP PRECURSOR

PDB ID 1ivy

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