6bh8

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Current revision (14:40, 4 October 2023) (edit) (undo)
 
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<StructureSection load='6bh8' size='340' side='right'caption='[[6bh8]], [[Resolution|resolution]] 3.85&Aring;' scene=''>
<StructureSection load='6bh8' size='340' side='right'caption='[[6bh8]], [[Resolution|resolution]] 3.85&Aring;' scene=''>
== Structural highlights ==
== Structural highlights ==
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<table><tr><td colspan='2'>[[6bh8]] is a 2 chain structure with sequence from [http://en.wikipedia.org/wiki/Human Human]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=6BH8 OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=6BH8 FirstGlance]. <br>
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<table><tr><td colspan='2'>[[6bh8]] 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=6BH8 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=6BH8 FirstGlance]. <br>
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</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=C8E:(HYDROXYETHYLOXY)TRI(ETHYLOXY)OCTANE'>C8E</scene>, <scene name='pdbligand=RDF:N-ALPHA-L-RHAMNOPYRANOSYLOXY(HYDROXYPHOSPHINYL)-L-LEUCYL-L-TRYPTOPHAN'>RDF</scene>, <scene name='pdbligand=ZN:ZINC+ION'>ZN</scene></td></tr>
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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]] 3.85&#8491;</td></tr>
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<tr id='related'><td class="sblockLbl"><b>[[Related_structure|Related:]]</b></td><td class="sblockDat">[[5syt|5syt]], [[4aw6|4aw6]], [[2ypt|2ypt]]</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=C8E:(HYDROXYETHYLOXY)TRI(ETHYLOXY)OCTANE'>C8E</scene>, <scene name='pdbligand=RDF:N-ALPHA-L-RHAMNOPYRANOSYLOXY(HYDROXYPHOSPHINYL)-L-LEUCYL-L-TRYPTOPHAN'>RDF</scene>, <scene name='pdbligand=ZN:ZINC+ION'>ZN</scene></td></tr>
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<tr id='gene'><td class="sblockLbl"><b>[[Gene|Gene:]]</b></td><td class="sblockDat">ZMPSTE24, FACE1, STE24 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 HUMAN])</td></tr>
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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=6bh8 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6bh8 OCA], [https://pdbe.org/6bh8 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=6bh8 RCSB], [https://www.ebi.ac.uk/pdbsum/6bh8 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=6bh8 ProSAT]</span></td></tr>
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<tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Ste24_endopeptidase Ste24 endopeptidase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=3.4.24.84 3.4.24.84] </span></td></tr>
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<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=6bh8 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6bh8 OCA], [http://pdbe.org/6bh8 PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=6bh8 RCSB], [http://www.ebi.ac.uk/pdbsum/6bh8 PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=6bh8 ProSAT]</span></td></tr>
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</table>
</table>
== Disease ==
== Disease ==
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[[http://www.uniprot.org/uniprot/FACE1_HUMAN FACE1_HUMAN]] Mandibuloacral dysplasia with type B lipodystrophy;Hutchinson-Gilford progeria syndrome;Lethal restrictive dermopathy. Mandibuloacral dysplasia with type B lipodystrophy (MADB) [MIM:[http://omim.org/entry/608612 608612]]: A disorder characterized by mandibular and clavicular hypoplasia, acroosteolysis, delayed closure of the cranial suture, joint contractures, and generalized lipodystrophy with loss of subcutaneous fat from the extremities, face, neck and trunk. Note=The disease is caused by mutations affecting the gene represented in this entry.<ref>PMID:12913070</ref> <ref>PMID:17152860</ref> <ref>PMID:18435794</ref> <ref>PMID:20814950</ref> Lethal tight skin contracture syndrome (LTSCS) [MIM:[http://omim.org/entry/275210 275210]]: Rare disorder mainly characterized by intrauterine growth retardation, tight and rigid skin with erosions, prominent superficial vasculature and epidermal hyperkeratosis, facial features (small mouth, small pinched nose and micrognathia), sparse/absent eyelashes and eyebrows, mineralization defects of the skull, thin dysplastic clavicles, pulmonary hypoplasia, multiple joint contractures and an early neonatal lethal course. Liveborn children usually die within the first week of life. The overall prevalence of consanguineous cases suggested an autosomal recessive inheritance. Note=The disease is caused by mutations affecting the gene represented in this entry.<ref>PMID:15317753</ref>
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[https://www.uniprot.org/uniprot/FACE1_HUMAN FACE1_HUMAN] Mandibuloacral dysplasia with type B lipodystrophy;Hutchinson-Gilford progeria syndrome;Lethal restrictive dermopathy. Mandibuloacral dysplasia with type B lipodystrophy (MADB) [MIM:[https://omim.org/entry/608612 608612]: A disorder characterized by mandibular and clavicular hypoplasia, acroosteolysis, delayed closure of the cranial suture, joint contractures, and generalized lipodystrophy with loss of subcutaneous fat from the extremities, face, neck and trunk. Note=The disease is caused by mutations affecting the gene represented in this entry.<ref>PMID:12913070</ref> <ref>PMID:17152860</ref> <ref>PMID:18435794</ref> <ref>PMID:20814950</ref> Lethal tight skin contracture syndrome (LTSCS) [MIM:[https://omim.org/entry/275210 275210]: Rare disorder mainly characterized by intrauterine growth retardation, tight and rigid skin with erosions, prominent superficial vasculature and epidermal hyperkeratosis, facial features (small mouth, small pinched nose and micrognathia), sparse/absent eyelashes and eyebrows, mineralization defects of the skull, thin dysplastic clavicles, pulmonary hypoplasia, multiple joint contractures and an early neonatal lethal course. Liveborn children usually die within the first week of life. The overall prevalence of consanguineous cases suggested an autosomal recessive inheritance. Note=The disease is caused by mutations affecting the gene represented in this entry.<ref>PMID:15317753</ref>
== Function ==
== Function ==
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[[http://www.uniprot.org/uniprot/FACE1_HUMAN FACE1_HUMAN]] Proteolytically removes the C-terminal three residues of farnesylated proteins. Acts on lamin A/C.
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[https://www.uniprot.org/uniprot/FACE1_HUMAN FACE1_HUMAN] Proteolytically removes the C-terminal three residues of farnesylated proteins. Acts on lamin A/C.
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<div style="background-color:#fffaf0;">
== Publication Abstract from PubMed ==
== Publication Abstract from PubMed ==
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__TOC__
__TOC__
</StructureSection>
</StructureSection>
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[[Category: Human]]
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[[Category: Homo sapiens]]
[[Category: Large Structures]]
[[Category: Large Structures]]
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[[Category: Ste24 endopeptidase]]
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[[Category: Arachea BT]]
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[[Category: Arachea, B T]]
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[[Category: Goblirsch BR]]
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[[Category: Goblirsch, B R]]
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[[Category: Wiener MC]]
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[[Category: Wiener, M C]]
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[[Category: Hydrolase]]
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[[Category: Inhibitor complex]]
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[[Category: Integral membrane protein]]
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[[Category: Zinc metalloprotease]]
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Current revision

Crystal structure of ZMPSTE24 in complex with phosphoramidon

PDB ID 6bh8

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