1pv8: Difference between revisions
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== Structural highlights == | == Structural highlights == | ||
<table><tr><td colspan='2'>[[1pv8]] is a 2 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=1PV8 OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=1PV8 FirstGlance]. <br> | <table><tr><td colspan='2'>[[1pv8]] is a 2 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=1PV8 OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=1PV8 FirstGlance]. <br> | ||
</td></tr><tr><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=PB1:3-(2-AMINOETHYL)-4-(AMINOMETHYL)HEPTANEDIOIC+ACID'>PB1</scene>, <scene name='pdbligand=ZN:ZINC+ION'>ZN</scene>< | </td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=PB1:3-(2-AMINOETHYL)-4-(AMINOMETHYL)HEPTANEDIOIC+ACID'>PB1</scene>, <scene name='pdbligand=ZN:ZINC+ION'>ZN</scene></td></tr> | ||
<tr><td class="sblockLbl"><b>[[Gene|Gene:]]</b></td><td class="sblockDat">ALAD ([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">ALAD ([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/Porphobilinogen_synthase Porphobilinogen synthase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=4.2.1.24 4.2.1.24] </span></td></tr> | <tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Porphobilinogen_synthase Porphobilinogen synthase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=4.2.1.24 4.2.1.24] </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=1pv8 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1pv8 OCA], [http://www.rcsb.org/pdb/explore.do?structureId=1pv8 RCSB], [http://www.ebi.ac.uk/pdbsum/1pv8 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=1pv8 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1pv8 OCA], [http://www.rcsb.org/pdb/explore.do?structureId=1pv8 RCSB], [http://www.ebi.ac.uk/pdbsum/1pv8 PDBsum]</span></td></tr> | ||
<table> | </table> | ||
== Disease == | == Disease == | ||
[[http://www.uniprot.org/uniprot/HEM2_HUMAN HEM2_HUMAN]] Defects in ALAD are the cause of acute hepatic porphyria (AHEPP) [MIM:[http://omim.org/entry/612740 612740]]. A form of porphyria. Porphyrias are inherited defects in the biosynthesis of heme, resulting in the accumulation and increased excretion of porphyrins or porphyrin precursors. They are classified as erythropoietic or hepatic, depending on whether the enzyme deficiency occurs in red blood cells or in the liver. AHP is characterized by attacks of gastrointestinal disturbances, abdominal colic, paralysis, and peripheral neuropathy. Most attacks are precipitated by drugs, alcohol, caloric deprivation, infections, or endocrine factors.<ref>PMID:1569184</ref> <ref>PMID:2063868</ref> <ref>PMID:1309003</ref> <ref>PMID:10706561</ref> <ref>PMID:17236137</ref> | [[http://www.uniprot.org/uniprot/HEM2_HUMAN HEM2_HUMAN]] Defects in ALAD are the cause of acute hepatic porphyria (AHEPP) [MIM:[http://omim.org/entry/612740 612740]]. A form of porphyria. Porphyrias are inherited defects in the biosynthesis of heme, resulting in the accumulation and increased excretion of porphyrins or porphyrin precursors. They are classified as erythropoietic or hepatic, depending on whether the enzyme deficiency occurs in red blood cells or in the liver. AHP is characterized by attacks of gastrointestinal disturbances, abdominal colic, paralysis, and peripheral neuropathy. Most attacks are precipitated by drugs, alcohol, caloric deprivation, infections, or endocrine factors.<ref>PMID:1569184</ref> <ref>PMID:2063868</ref> <ref>PMID:1309003</ref> <ref>PMID:10706561</ref> <ref>PMID:17236137</ref> | ||
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[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
[[Category: Porphobilinogen synthase]] | [[Category: Porphobilinogen synthase]] | ||
[[Category: Breinig, S | [[Category: Breinig, S]] | ||
[[Category: Fairman, R | [[Category: Fairman, R]] | ||
[[Category: Jaffe, E K | [[Category: Jaffe, E K]] | ||
[[Category: Kervinen, J | [[Category: Kervinen, J]] | ||
[[Category: Stith, L | [[Category: Stith, L]] | ||
[[Category: Wasson, A S | [[Category: Wasson, A S]] | ||
[[Category: Wlodawer, A | [[Category: Wlodawer, A]] | ||
[[Category: Zdanov, A | [[Category: Zdanov, A]] | ||
[[Category: Lyase]] | [[Category: Lyase]] | ||
[[Category: Reaction intermediate]] | [[Category: Reaction intermediate]] | ||
[[Category: Tetrapyrrole biosynthesis]] | [[Category: Tetrapyrrole biosynthesis]] | ||