1ivh
From Proteopedia
(Difference between revisions)
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== Structural highlights == | == Structural highlights == | ||
<table><tr><td colspan='2'>[[1ivh]] 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=1IVH OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=1IVH FirstGlance]. <br> | <table><tr><td colspan='2'>[[1ivh]] 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=1IVH OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=1IVH FirstGlance]. <br> | ||
| - | </td></tr><tr><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=COS:COENZYME+A+PERSULFIDE'>COS</scene>, <scene name='pdbligand=FAD:FLAVIN-ADENINE+DINUCLEOTIDE'>FAD</scene>< | + | </td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=COS:COENZYME+A+PERSULFIDE'>COS</scene>, <scene name='pdbligand=FAD:FLAVIN-ADENINE+DINUCLEOTIDE'>FAD</scene></td></tr> |
| - | <tr><td class="sblockLbl"><b>[[Gene|Gene:]]</b></td><td class="sblockDat">IVD ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 Homo sapiens])</td></tr> | + | <tr id='gene'><td class="sblockLbl"><b>[[Gene|Gene:]]</b></td><td class="sblockDat">IVD ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 Homo sapiens])</td></tr> |
| - | <tr><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Isovaleryl-CoA_dehydrogenase Isovaleryl-CoA dehydrogenase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=1.3.99.10 1.3.99.10] </span></td></tr> | + | <tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Isovaleryl-CoA_dehydrogenase Isovaleryl-CoA dehydrogenase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=1.3.99.10 1.3.99.10] </span></td></tr> |
| - | <tr><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=1ivh FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1ivh OCA], [http://www.rcsb.org/pdb/explore.do?structureId=1ivh RCSB], [http://www.ebi.ac.uk/pdbsum/1ivh PDBsum]</span></td></tr> | + | <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=1ivh FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1ivh OCA], [http://www.rcsb.org/pdb/explore.do?structureId=1ivh RCSB], [http://www.ebi.ac.uk/pdbsum/1ivh PDBsum]</span></td></tr> |
| - | <table> | + | </table> |
== Disease == | == Disease == | ||
[[http://www.uniprot.org/uniprot/IVD_HUMAN IVD_HUMAN]] Defects in IVD are the cause of isovaleric acidemia (IVA) [MIM:[http://omim.org/entry/243500 243500]]. IVA is characterized by retarded psychomotor development, a peculiar odor resembling sweaty feet, an aversion to dietary protein, and pernicious vomiting, leading to acidosis and coma. The acute neonatal form leads to massive metabolic acidosis from the first days of life and rapid death.<ref>PMID:2063866</ref> <ref>PMID:9665741</ref> | [[http://www.uniprot.org/uniprot/IVD_HUMAN IVD_HUMAN]] Defects in IVD are the cause of isovaleric acidemia (IVA) [MIM:[http://omim.org/entry/243500 243500]]. IVA is characterized by retarded psychomotor development, a peculiar odor resembling sweaty feet, an aversion to dietary protein, and pernicious vomiting, leading to acidosis and coma. The acute neonatal form leads to massive metabolic acidosis from the first days of life and rapid death.<ref>PMID:2063866</ref> <ref>PMID:9665741</ref> | ||
| - | == Function == | ||
| - | |||
== Evolutionary Conservation == | == Evolutionary Conservation == | ||
[[Image:Consurf_key_small.gif|200px|right]] | [[Image:Consurf_key_small.gif|200px|right]] | ||
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[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
[[Category: Isovaleryl-CoA dehydrogenase]] | [[Category: Isovaleryl-CoA dehydrogenase]] | ||
| - | [[Category: Kim, J J.P | + | [[Category: Kim, J J.P]] |
| - | [[Category: Mohsen, A W.A | + | [[Category: Mohsen, A W.A]] |
| - | [[Category: Paschke, R | + | [[Category: Paschke, R]] |
| - | [[Category: Roberts, D L | + | [[Category: Roberts, D L]] |
| - | [[Category: Tiffany, K A | + | [[Category: Tiffany, K A]] |
| - | [[Category: Vockley, J | + | [[Category: Vockley, J]] |
| - | [[Category: Wang, M | + | [[Category: Wang, M]] |
[[Category: Acyl-coa dehydrogenase]] | [[Category: Acyl-coa dehydrogenase]] | ||
[[Category: Flavoprotein]] | [[Category: Flavoprotein]] | ||
Revision as of 21:51, 25 December 2014
STRUCTURE OF HUMAN ISOVALERYL-COA DEHYDROGENASE AT 2.6 ANGSTROMS RESOLUTION: STRUCTURAL BASIS FOR SUBSTRATE SPECIFICITY
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