1fch: Difference between revisions

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-{{Seed}}
-[[Image:1fch.png|left|200px]]
-<!--
+==CRYSTAL STRUCTURE OF THE PTS1 COMPLEXED TO THE TPR REGION OF HUMAN PEX5==
-The line below this paragraph, containing "STRUCTURE_1fch", creates the "Structure Box" on the page.
+<StructureSection load='1fch' size='340' side='right'caption='[[1fch]], [[Resolution|resolution]] 2.20&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'>[[1fch]] is a 4 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=1FCH OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=1FCH 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]] 2.2&#8491;</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=1fch FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1fch OCA], [https://pdbe.org/1fch PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=1fch RCSB], [https://www.ebi.ac.uk/pdbsum/1fch PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=1fch ProSAT]</span></td></tr>
-{{STRUCTURE_1fch|  PDB=1fch  |  SCENE=  }}
+</table>
+== Disease ==
-===CRYSTAL STRUCTURE OF THE PTS1 COMPLEXED TO THE TPR REGION OF HUMAN PEX5===
+[https://www.uniprot.org/uniprot/PEX5_HUMAN PEX5_HUMAN] Defects in PEX5 are the cause of peroxisome biogenesis disorder 2A (PBD2A) [MIM:[https://omim.org/entry/214110 214110]. A fatal peroxisome biogenesis disorder belonging to the Zellweger disease spectrum and characterized clinically by severe neurologic dysfunction with profound psychomotor retardation, severe hypotonia and neonatal seizures, craniofacial abnormalities, liver dysfunction, and biochemically by the absence of peroxisomes. Additional features include cardiovascular and skeletal defects, renal cysts, ocular abnormalities, and hearing impairment. Most severely affected individuals with the classic form of the disease (classic Zellweger syndrome) die within the first year of life.<ref>PMID:7719337</ref>   Defects in PEX5 are the cause of peroxisome biogenesis disorder 2B (PBD2B) [MIM:[https://omim.org/entry/202370 202370]. A peroxisome biogenesis disorder that includes neonatal adrenoleukodystrophy (NALD) and infantile Refsum disease (IRD), two milder manifestations of the Zellweger disease spectrum. The clinical course of patients with the NALD and IRD presentation is variable and may include developmental delay, hypotonia, liver dysfunction, sensorineural hearing loss, retinal dystrophy and vision impairment. Children with the NALD presentation may reach their teens, while patients with the IRD presentation may reach adulthood. The clinical conditions are often slowly progressive in particular with respect to loss of hearing and vision. The biochemical abnormalities include accumulation of phytanic acid, very long chain fatty acids (VLCFA), di- and trihydroxycholestanoic acid and pipecolic acid.
+== Function ==
+[https://www.uniprot.org/uniprot/PEX5_HUMAN PEX5_HUMAN] Binds to the C-terminal PTS1-type tripeptide peroxisomal targeting signal (SKL-type) and plays an essential role in peroxisomal protein import.<ref>PMID:7719337</ref> <ref>PMID:7790377</ref> <ref>PMID:7706321</ref>
-<!--
+== Evolutionary Conservation ==
-The line below this paragraph, {{ABSTRACT_PUBMED_11101887}}, adds the Publication Abstract to the page
+[[Image:Consurf_key_small.gif|200px|right]]
-(as it appears on PubMed at http://www.pubmed.gov), where 11101887 is the PubMed ID number.
+Check<jmol>
--->
+  <jmolCheckbox>
-{{ABSTRACT_PUBMED_11101887}}
+    <scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/fc/1fch_consurf.spt"</scriptWhenChecked>
+    <scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked>
-==About this Structure==
+    <text>to colour the structure by Evolutionary Conservation</text>
-FCH 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=1FCH OCA].
+  </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=1fch ConSurf].
-==Reference==
+<div style="clear:both"></div>
-Peroxisomal targeting signal-1 recognition by the TPR domains of human PEX5., Gatto GJ Jr, Geisbrecht BV, Gould SJ, Berg JM, Nat Struct Biol. 2000 Dec;7(12):1091-5. PMID:[http://www.ncbi.nlm.nih.gov/pubmed/11101887 11101887]
+== References ==
+<references/>
+__TOC__
+</StructureSection>
 [[Category: Homo sapiens]]
-[[Category: Single protein]]
+[[Category: Large Structures]]
-[[Category: Berg, J M.]]
+[[Category: Berg JM]]
-[[Category: Geisbrecht, B V.]]
+[[Category: Gatto Jr GJ]]
-[[Category: Gould, S J.]]
+[[Category: Geisbrecht BV]]
-[[Category: Jr., G J.Gatto.]]
+[[Category: Gould SJ]]
-[[Category: Helical repeat]]
-[[Category: Protein-peptide complex]]
-[[Category: Tetratricopeptide repeat]]
-[[Category: Tpr]]
-''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Tue Jul  1 03:02:52 2008''