3kfq
From Proteopedia
(Difference between revisions)
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| - | + | ==Unreduced cathepsin V in complex with stefin A== | |
| - | === | + | <StructureSection load='3kfq' size='340' side='right' caption='[[3kfq]], [[Resolution|resolution]] 1.99Å' scene=''> |
| - | + | == Structural highlights == | |
| - | ==Disease== | + | <table><tr><td colspan='2'>[[3kfq]] is a 4 chain structure with 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=3KFQ OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=3KFQ FirstGlance]. <br> |
| - | [[http://www.uniprot.org/uniprot/CYTA_HUMAN CYTA_HUMAN]] Defects in CSTA are the cause of ichthyosis exfoliative autosomal recessive ichthyosis bullosa of Siemens-like (AREI) [MIM:[http://omim.org/entry/607936 607936]]. A form of congenital exfoliative ichthyosis, sharing some features with ichthyosis bullosa of Siemens and annular epidermolytic ichthyosis. AREI presents shortly after birth as dry, scaly skin over most of the body with coarse peeling of non-erythematous skin on the palms and soles, which is exacerbated by excessive moisture and minor trauma. Electron microscopy analysis of skin biopsies, reveals mostly normal-appearing upper layers of the epidermis, but prominent intercellular edema of the basal and suprabasal cell layers with aggregates of tonofilaments in the basal keratinocytes.<ref>PMID:21944047</ref> | + | </td></tr><tr id='NonStdRes'><td class="sblockLbl"><b>[[Non-Standard_Residue|NonStd Res:]]</b></td><td class="sblockDat"><scene name='pdbligand=SCH:S-METHYL-THIO-CYSTEINE'>SCH</scene></td></tr> |
| - | + | <tr id='related'><td class="sblockLbl"><b>[[Related_structure|Related:]]</b></td><td class="sblockDat">[[1nb3|1nb3]], [[1stf|1stf]], [[3k9m|3k9m]], [[3a9n|3a9n]]</td></tr> | |
| - | ==Function== | + | <tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Cathepsin_V Cathepsin V], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=3.4.22.43 3.4.22.43] </span></td></tr> |
| - | [[http://www.uniprot.org/uniprot/CATL2_HUMAN CATL2_HUMAN]] Cysteine protease. May have an important role in corneal physiology.<ref>PMID:9727401</ref><ref>PMID:10029531</ref> [[http://www.uniprot.org/uniprot/CYTA_HUMAN CYTA_HUMAN]] This is an intracellular thiol proteinase inhibitor. Has an important role in desmosome-mediated cell-cell adhesion in the lower levels of the epidermis.<ref>PMID:21944047</ref> | + | <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=3kfq FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3kfq OCA], [http://www.rcsb.org/pdb/explore.do?structureId=3kfq RCSB], [http://www.ebi.ac.uk/pdbsum/3kfq PDBsum]</span></td></tr> |
| - | + | </table> | |
| - | + | == Disease == | |
| - | + | [[http://www.uniprot.org/uniprot/CYTA_HUMAN CYTA_HUMAN]] Defects in CSTA are the cause of ichthyosis exfoliative autosomal recessive ichthyosis bullosa of Siemens-like (AREI) [MIM:[http://omim.org/entry/607936 607936]]. A form of congenital exfoliative ichthyosis, sharing some features with ichthyosis bullosa of Siemens and annular epidermolytic ichthyosis. AREI presents shortly after birth as dry, scaly skin over most of the body with coarse peeling of non-erythematous skin on the palms and soles, which is exacerbated by excessive moisture and minor trauma. Electron microscopy analysis of skin biopsies, reveals mostly normal-appearing upper layers of the epidermis, but prominent intercellular edema of the basal and suprabasal cell layers with aggregates of tonofilaments in the basal keratinocytes.<ref>PMID:21944047</ref> | |
| + | == Function == | ||
| + | [[http://www.uniprot.org/uniprot/CATL2_HUMAN CATL2_HUMAN]] Cysteine protease. May have an important role in corneal physiology.<ref>PMID:9727401</ref> <ref>PMID:10029531</ref> [[http://www.uniprot.org/uniprot/CYTA_HUMAN CYTA_HUMAN]] This is an intracellular thiol proteinase inhibitor. Has an important role in desmosome-mediated cell-cell adhesion in the lower levels of the epidermis.<ref>PMID:21944047</ref> | ||
==See Also== | ==See Also== | ||
*[[Cathepsin|Cathepsin]] | *[[Cathepsin|Cathepsin]] | ||
| - | + | == References == | |
| - | == | + | <references/> |
| - | <references | + | __TOC__ |
| + | </StructureSection> | ||
[[Category: Cathepsin V]] | [[Category: Cathepsin V]] | ||
[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
| - | [[Category: Renko, M | + | [[Category: Renko, M]] |
| - | [[Category: Turk, D | + | [[Category: Turk, D]] |
[[Category: Cathepsin]] | [[Category: Cathepsin]] | ||
[[Category: Hydrolase]] | [[Category: Hydrolase]] | ||
Revision as of 17:21, 18 December 2014
Unreduced cathepsin V in complex with stefin A
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