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[[Image:1wsr.gif|left|200px]]


{{Structure
==Crystal Structure of Human T-protein of Glycine Cleavage System==
|PDB= 1wsr |SIZE=350|CAPTION= <scene name='initialview01'>1wsr</scene>, resolution 2.00&Aring;
<StructureSection load='1wsr' size='340' side='right'caption='[[1wsr]], [[Resolution|resolution]] 2.00&Aring;' scene=''>
|SITE=  
== Structural highlights ==
|LIGAND= <scene name='pdbligand=SO4:SULFATE ION'>SO4</scene>
<table><tr><td colspan='2'>[[1wsr]] is a 2 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=1WSR OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=1WSR FirstGlance]. <br>
|ACTIVITY= [http://en.wikipedia.org/wiki/Aminomethyltransferase Aminomethyltransferase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=2.1.2.10 2.1.2.10]  
</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&#8491;</td></tr>
|GENE= GCST ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 Homo sapiens])
<tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene></td></tr>
}}
<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=1wsr FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1wsr OCA], [https://pdbe.org/1wsr PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=1wsr RCSB], [https://www.ebi.ac.uk/pdbsum/1wsr PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=1wsr ProSAT]</span></td></tr>
</table>
== Disease ==
[https://www.uniprot.org/uniprot/GCST_HUMAN GCST_HUMAN] Defects in AMT are a cause of non-ketotic hyperglycinemia (NKH) [MIM:[https://omim.org/entry/605899 605899]; also known as glycine encephalopathy (GCE). NKH is an autosomal recessive disease characterized by accumulation of a large amount of glycine in body fluid and by severe neurological symptoms.<ref>PMID:8005589</ref> <ref>PMID:9600239</ref> <ref>PMID:9621520</ref> <ref>PMID:10873393</ref> <ref>PMID:11286506</ref>
== Function ==
[https://www.uniprot.org/uniprot/GCST_HUMAN GCST_HUMAN] The glycine cleavage system catalyzes the degradation of glycine.
== Evolutionary Conservation ==
[[Image:Consurf_key_small.gif|200px|right]]
Check<jmol>
  <jmolCheckbox>
    <scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/ws/1wsr_consurf.spt"</scriptWhenChecked>
    <scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked>
    <text>to colour the structure by Evolutionary Conservation</text>
  </jmolCheckbox>
</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=1wsr ConSurf].
<div style="clear:both"></div>


'''Crystal Structure of Human T-protein of Glycine Cleavage System'''
==See Also==
 
*[[Aminomethyltransferase 3D structures|Aminomethyltransferase 3D structures]]
 
== References ==
==Overview==
<references/>
T-protein, a component of the glycine cleavage system, catalyzes the formation of ammonia and 5,10-methylenetetrahydrofolate from the aminomethyl moiety of glycine attached to the lipoate cofactor of H-protein. Several mutations in the human T-protein gene cause non-ketotic hyperglycinemia. To gain insights into the effect of disease-causing mutations and the catalytic mechanism at the molecular level, crystal structures of human T-protein in free form and that bound to 5-methyltetrahydrofolate (5-CH3-H4folate) have been determined at 2.0 A and 2.6 A resolution, respectively. The overall structure consists of three domains arranged in a cloverleaf-like structure with the central cavity, where 5-CH3-H4folate is bound in a kinked shape with the pteridine group deeply buried into the hydrophobic pocket and the glutamyl group pointed to the C-terminal side surface. Most of the disease-related residues cluster around the cavity, forming extensive hydrogen bonding networks. These hydrogen bonding networks are employed in holding not only the folate-binding space but also the positions and the orientations of alpha-helix G and the following loop in the middle region, which seems to play a pivotal role in the T-protein catalysis. Structural and mutational analyses demonstrated that Arg292 interacts through water molecules with the folate polyglutamate tail, and that the invariant Asp101, located close to the N10 group of 5-CH3-H4folate, might play a key role in the initiation of the catalysis by increasing the nucleophilic character of the N10 atom of the folate substrate for the nucleophilic attack on the aminomethyl lipoate intermediate. A clever mechanism of recruiting the aminomethyl lipoate arm to the reaction site seems to function as a way of avoiding the release of toxic formaldehyde.
__TOC__
 
</StructureSection>
==Disease==
Known disease associated with this structure: Glycine encephalopathy OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=238310 238310]]
 
==About this Structure==
1WSR 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=1WSR OCA].
 
==Reference==
Crystal structure of human T-protein of glycine cleavage system at 2.0 A resolution and its implication for understanding non-ketotic hyperglycinemia., Okamura-Ikeda K, Hosaka H, Yoshimura M, Yamashita E, Toma S, Nakagawa A, Fujiwara K, Motokawa Y, Taniguchi H, J Mol Biol. 2005 Sep 2;351(5):1146-59. PMID:[http://www.ncbi.nlm.nih.gov/pubmed/16051266 16051266]
[[Category: Aminomethyltransferase]]
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
[[Category: Single protein]]
[[Category: Large Structures]]
[[Category: Fujiwara, K.]]
[[Category: Fujiwara K]]
[[Category: Hosaka, H.]]
[[Category: Hosaka H]]
[[Category: Motokawa, Y.]]
[[Category: Motokawa Y]]
[[Category: Nakagawa, A.]]
[[Category: Nakagawa A]]
[[Category: Okamura-Ikeda, K.]]
[[Category: Okamura-Ikeda K]]
[[Category: Taniguchi, H.]]
[[Category: Taniguchi H]]
[[Category: Toma, S.]]
[[Category: Toma S]]
[[Category: Yamashita, E.]]
[[Category: Yamashita E]]
[[Category: Yoshimura, M.]]
[[Category: Yoshimura M]]
[[Category: SO4]]
[[Category: aminomethyl transferase]]
[[Category: glycine-cleavage sytem]]
 
''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu Mar 20 15:01:03 2008''
Retrieved from "https://proteopedia.org/1wsr"