2vd6
From Proteopedia
(Difference between revisions)
| Line 3: | Line 3: | ||
== Structural highlights == | == Structural highlights == | ||
<table><tr><td colspan='2'>[[2vd6]] 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=2VD6 OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=2VD6 FirstGlance]. <br> | <table><tr><td colspan='2'>[[2vd6]] 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=2VD6 OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=2VD6 FirstGlance]. <br> | ||
| - | </td></tr><tr><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=2SA:2-[9-(3,4-DIHYDROXY-5-PHOSPHONOOXYMETHYL-TETRAHYDRO-FURAN-2-YL)-9H-PURIN-6-YLAMINO]-SUCCINIC+ACID'>2SA</scene>, <scene name='pdbligand=AMP:ADENOSINE+MONOPHOSPHATE'>AMP</scene>, <scene name='pdbligand=CL:CHLORIDE+ION'>CL</scene>, <scene name='pdbligand=FUM:FUMARIC+ACID'>FUM</scene>, <scene name='pdbligand=GOL:GLYCEROL'>GOL</scene>< | + | </td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=2SA:2-[9-(3,4-DIHYDROXY-5-PHOSPHONOOXYMETHYL-TETRAHYDRO-FURAN-2-YL)-9H-PURIN-6-YLAMINO]-SUCCINIC+ACID'>2SA</scene>, <scene name='pdbligand=AMP:ADENOSINE+MONOPHOSPHATE'>AMP</scene>, <scene name='pdbligand=CL:CHLORIDE+ION'>CL</scene>, <scene name='pdbligand=FUM:FUMARIC+ACID'>FUM</scene>, <scene name='pdbligand=GOL:GLYCEROL'>GOL</scene></td></tr> |
| - | <tr><td class="sblockLbl"><b>[[Related_structure|Related:]]</b></td><td class="sblockDat">[[2j91|2j91]]</td></tr> | + | <tr id='related'><td class="sblockLbl"><b>[[Related_structure|Related:]]</b></td><td class="sblockDat">[[2j91|2j91]]</td></tr> |
| - | <tr><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Adenylosuccinate_lyase Adenylosuccinate lyase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=4.3.2.2 4.3.2.2] </span></td></tr> | + | <tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Adenylosuccinate_lyase Adenylosuccinate lyase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=4.3.2.2 4.3.2.2] </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=2vd6 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2vd6 OCA], [http://www.rcsb.org/pdb/explore.do?structureId=2vd6 RCSB], [http://www.ebi.ac.uk/pdbsum/2vd6 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=2vd6 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2vd6 OCA], [http://www.rcsb.org/pdb/explore.do?structureId=2vd6 RCSB], [http://www.ebi.ac.uk/pdbsum/2vd6 PDBsum]</span></td></tr> |
| - | <table> | + | </table> |
== Disease == | == Disease == | ||
[[http://www.uniprot.org/uniprot/PUR8_HUMAN PUR8_HUMAN]] Defects in ADSL are the cause of adenylosuccinase deficiency (ADSL deficiency) [MIM:[http://omim.org/entry/103050 103050]]. ADSL deficiency is an autosomal recessive disorder characterized by the accumulation in the body fluids of succinylaminoimidazole-carboxamide riboside (SAICA-riboside) and succinyladenosine (S-Ado). Most children display marked psychomotor delay, often accompanied by epilepsy or autistic features, or both, although some patients may be less profoundly retarded. Occasionally, growth retardation and muscular wasting are also present. | [[http://www.uniprot.org/uniprot/PUR8_HUMAN PUR8_HUMAN]] Defects in ADSL are the cause of adenylosuccinase deficiency (ADSL deficiency) [MIM:[http://omim.org/entry/103050 103050]]. ADSL deficiency is an autosomal recessive disorder characterized by the accumulation in the body fluids of succinylaminoimidazole-carboxamide riboside (SAICA-riboside) and succinyladenosine (S-Ado). Most children display marked psychomotor delay, often accompanied by epilepsy or autistic features, or both, although some patients may be less profoundly retarded. Occasionally, growth retardation and muscular wasting are also present. | ||
| - | == Function == | ||
| - | |||
== Evolutionary Conservation == | == Evolutionary Conservation == | ||
[[Image:Consurf_key_small.gif|200px|right]] | [[Image:Consurf_key_small.gif|200px|right]] | ||
| Line 26: | Line 24: | ||
[[Category: Adenylosuccinate lyase]] | [[Category: Adenylosuccinate lyase]] | ||
[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
| - | [[Category: Arrowsmith, C | + | [[Category: Arrowsmith, C]] |
| - | [[Category: Berg, S Van Den | + | [[Category: Berg, S Van Den]] |
| - | [[Category: Berglund, H | + | [[Category: Berglund, H]] |
| - | [[Category: Busam, R | + | [[Category: Busam, R]] |
| - | [[Category: Collins, R | + | [[Category: Collins, R]] |
| - | [[Category: Dahlgren, L G | + | [[Category: Dahlgren, L G]] |
| - | [[Category: Edwards, A | + | [[Category: Edwards, A]] |
| - | [[Category: Flodin, S | + | [[Category: Flodin, S]] |
| - | [[Category: Flores, A | + | [[Category: Flores, A]] |
| - | [[Category: Graslund, S | + | [[Category: Graslund, S]] |
| - | [[Category: Hallberg, B M | + | [[Category: Hallberg, B M]] |
| - | [[Category: Hammarstrom, M | + | [[Category: Hammarstrom, M]] |
| - | [[Category: Holmberg-Schiavone, L | + | [[Category: Holmberg-Schiavone, L]] |
| - | [[Category: Johansson, I | + | [[Category: Johansson, I]] |
| - | [[Category: Kallas, A | + | [[Category: Kallas, A]] |
| - | [[Category: Karlberg, T | + | [[Category: Karlberg, T]] |
| - | [[Category: Kotenyova, T | + | [[Category: Kotenyova, T]] |
| - | [[Category: Lehtio, L | + | [[Category: Lehtio, L]] |
| - | [[Category: Moche, M | + | [[Category: Moche, M]] |
| - | [[Category: Nilsson, M | + | [[Category: Nilsson, M]] |
| - | [[Category: Nordlund, P | + | [[Category: Nordlund, P]] |
| - | [[Category: Nyman, T | + | [[Category: Nyman, T]] |
| - | [[Category: Ogg, D | + | [[Category: Ogg, D]] |
| - | [[Category: Persson, C | + | [[Category: Persson, C]] |
| - | [[Category: Sagemark, J | + | [[Category: Sagemark, J]] |
| - | [[Category: | + | [[Category: Structural genomic]] |
| - | [[Category: Stenmark, P | + | [[Category: Stenmark, P]] |
| - | [[Category: Sundstrom, M | + | [[Category: Sundstrom, M]] |
| - | [[Category: Thorsell, A G | + | [[Category: Thorsell, A G]] |
| - | [[Category: Tresaugues, L | + | [[Category: Tresaugues, L]] |
| - | [[Category: Weigelt, J | + | [[Category: Weigelt, J]] |
| - | [[Category: Welin, M | + | [[Category: Welin, M]] |
[[Category: Epilepsy]] | [[Category: Epilepsy]] | ||
[[Category: Lyase]] | [[Category: Lyase]] | ||
[[Category: Purine biosynthesis]] | [[Category: Purine biosynthesis]] | ||
[[Category: Purine metabolism]] | [[Category: Purine metabolism]] | ||
Revision as of 18:47, 24 December 2014
Human adenylosuccinate lyase in complex with its substrate N6-(1,2- Dicarboxyethyl)-AMP, and its products AMP and fumarate.
| |||||||||||
Categories: Adenylosuccinate lyase | Homo sapiens | Arrowsmith, C | Berg, S Van Den | Berglund, H | Busam, R | Collins, R | Dahlgren, L G | Edwards, A | Flodin, S | Flores, A | Graslund, S | Hallberg, B M | Hammarstrom, M | Holmberg-Schiavone, L | Johansson, I | Kallas, A | Karlberg, T | Kotenyova, T | Lehtio, L | Moche, M | Nilsson, M | Nordlund, P | Nyman, T | Ogg, D | Persson, C | Sagemark, J | Structural genomic | Stenmark, P | Sundstrom, M | Thorsell, A G | Tresaugues, L | Weigelt, J | Welin, M | Epilepsy | Lyase | Purine biosynthesis | Purine metabolism
