1m54

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[[Image:1m54.png|left|200px]]
 
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==CYSTATHIONINE-BETA SYNTHASE: REDUCED VICINAL THIOLS==
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The line below this paragraph, containing "STRUCTURE_1m54", creates the "Structure Box" on the page.
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<StructureSection load='1m54' size='340' side='right'caption='[[1m54]], [[Resolution|resolution]] 2.90&Aring;' scene=''>
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== Structural highlights ==
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or the SCENE parameter (which sets the initial scene displayed when the page is loaded),
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<table><tr><td colspan='2'>[[1m54]] is a 6 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=1M54 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=1M54 FirstGlance]. <br>
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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]] 2.9&#8491;</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=HEM:PROTOPORPHYRIN+IX+CONTAINING+FE'>HEM</scene>, <scene name='pdbligand=PLP:PYRIDOXAL-5-PHOSPHATE'>PLP</scene></td></tr>
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{{STRUCTURE_1m54| PDB=1m54 | SCENE= }}
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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=1m54 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1m54 OCA], [https://pdbe.org/1m54 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=1m54 RCSB], [https://www.ebi.ac.uk/pdbsum/1m54 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=1m54 ProSAT]</span></td></tr>
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</table>
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== Disease ==
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[https://www.uniprot.org/uniprot/CBS_HUMAN CBS_HUMAN] Defects in CBS are the cause of cystathionine beta-synthase deficiency (CBSD) [MIM:[https://omim.org/entry/236200 236200]. CBSD is an enzymatic deficiency resulting in altered sulfur metabolism and homocystinuria. The clinical features of untreated homocystinuria due to CBS deficiency include myopia, ectopia lentis, mental retardation, skeletal anomalies resembling Marfan syndrome, and thromboembolic events. Light skin and hair can also be present. Biochemical features include increased urinary homocystine and methionine.<ref>PMID:1301198</ref> <ref>PMID:8353501</ref> <ref>PMID:7506602</ref> <ref>PMID:7981678</ref> <ref>PMID:7849717</ref> <ref>PMID:7967489</ref> <ref>PMID:7611293</ref> <ref>PMID:7762555</ref> <ref>PMID:7635485</ref> <ref>PMID:8528202</ref> <ref>PMID:7564249</ref> <ref>PMID:8755636</ref> <ref>PMID:8803779</ref> <ref>PMID:9156316</ref> <ref>PMID:9361025</ref> <ref>PMID:8990018</ref> <ref>PMID:9266356</ref> <ref>PMID:10462600</ref> <ref>PMID:10215408</ref> <ref>PMID:9889017</ref> <ref>PMID:10408774</ref> <ref>PMID:11013450</ref> <ref>PMID:11359213</ref> <ref>PMID:11553052</ref> <ref>PMID:12007221</ref> <ref>PMID:12124992</ref> <ref>PMID:12815602</ref> <ref>PMID:14635102</ref> <ref>PMID:15146473</ref> <ref>PMID:15365998</ref> <ref>PMID:15993874</ref> <ref>PMID:16205833</ref> <ref>PMID:16429402</ref> <ref>PMID:21520339</ref> <ref>PMID:21240075</ref>
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== Function ==
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[https://www.uniprot.org/uniprot/CBS_HUMAN CBS_HUMAN] Only known pyridoxal phosphate-dependent enzyme that contains heme. Important regulator of hydrogen sulfide, especially in the brain, utilizing cysteine instead of serine to catalyze the formation of hydrogen sulfide. Hydrogen sulfide is a gastratransmitter with signaling and cytoprotective effects such as acting as a neuromodulator in the brain to protect neurons against hypoxic injury (By similarity).
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== Evolutionary Conservation ==
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[[Image:Consurf_key_small.gif|200px|right]]
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Check<jmol>
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<jmolCheckbox>
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<scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/m5/1m54_consurf.spt"</scriptWhenChecked>
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<scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked>
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<text>to colour the structure by Evolutionary Conservation</text>
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</jmolCheckbox>
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</jmol>, as determined by [http://consurfdb.tau.ac.il/ ConSurfDB]. You may read the [[Conservation%2C_Evolutionary|explanation]] of the method and the full data available from [http://bental.tau.ac.il/new_ConSurfDB/main_output.php?pdb_ID=1m54 ConSurf].
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<div style="clear:both"></div>
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===CYSTATHIONINE-BETA SYNTHASE: REDUCED VICINAL THIOLS===
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==See Also==
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*[[Cystathionine β-synthase 3D structures|Cystathionine β-synthase 3D structures]]
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== References ==
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<references/>
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The line below this paragraph, {{ABSTRACT_PUBMED_12173932}}, adds the Publication Abstract to the page
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__TOC__
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(as it appears on PubMed at http://www.pubmed.gov), where 12173932 is the PubMed ID number.
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</StructureSection>
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{{ABSTRACT_PUBMED_12173932}}
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==Disease==
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Known disease associated with this structure: Homocystinuria, B6-responsive and nonresponsive types OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=236200 236200]], Thrombosis, hyperhomocysteinemic OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=236200 236200]]
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==About this Structure==
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1M54 is a [[Single protein]] structure of 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=1M54 OCA].
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==Reference==
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Human cystathionine beta-synthase is a heme sensor protein. Evidence that the redox sensor is heme and not the vicinal cysteines in the CXXC motif seen in the crystal structure of the truncated enzyme., Taoka S, Lepore BW, Kabil O, Ojha S, Ringe D, Banerjee R, Biochemistry. 2002 Aug 20;41(33):10454-61. PMID:[http://www.ncbi.nlm.nih.gov/pubmed/12173932 12173932]
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[[Category: Cystathionine beta-synthase]]
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[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Single protein]]
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[[Category: Large Structures]]
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[[Category: Banerjee, R.]]
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[[Category: Banerjee R]]
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[[Category: Kabil, O.]]
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[[Category: Kabil O]]
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[[Category: Lepore, B W.]]
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[[Category: Lepore BW]]
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[[Category: Ojha, S.]]
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[[Category: Ojha S]]
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[[Category: Ringe, D.]]
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[[Category: Ringe D]]
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[[Category: Taoka, S.]]
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[[Category: Taoka S]]
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[[Category: Plp and heme bound to protein]]
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[[Category: Reduced vicinal cysteine]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Wed Jul 2 23:13:52 2008''
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Current revision

CYSTATHIONINE-BETA SYNTHASE: REDUCED VICINAL THIOLS

PDB ID 1m54

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