Ornithine carbamoyltransferase
From Proteopedia
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| - | <StructureSection load='2i6u' size=' | + | <StructureSection load='2i6u' size='450' side='right' caption='Structure of ornithine carbamoyltransferase complex with carbamoyl phosphate, sulfate and the competitive inhibitor norvaline (PDB entry [[2i6u]])' scene='57/573976/Cv/1'> |
== Function == | == Function == | ||
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== Disease == | == Disease == | ||
| - | + | OTC deficiency is a genetic urea cycle disorder<ref>PMID:23278509</ref>. | |
== Structural highlights == | == Structural highlights == | ||
| - | OTC structure contains an N-terminal - CP-binding domain and a C-terminal - ornithine binding domain. OCT active site is situated between the two domains<ref>PMID:18062991</ref>. | + | The biological assembly of OTC is <scene name='57/573976/Cv/6'>homotrimer</scene>. OTC structure contains an <scene name='57/573976/Cv/7'>N-terminal - CP-binding domain and a C-terminal - ornithine binding domain</scene>. OCT <scene name='57/573976/Cv/8'>active site is situated between the two domains</scene><ref>PMID:18062991</ref>. |
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==3D structures of ornithine carbamoyltransferase== | ==3D structures of ornithine carbamoyltransferase== | ||
| + | [[Ornithine carbamoyltransferase 3D structures]] | ||
| - | + | </StructureSection> | |
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| - | **[[1vlv]] – OTC + phosphate – ''Thermotoga maritima''<br /> | ||
| - | **[[2i6u]] – MtOTC + norvaline + carbamoyl phosphate <br /> | ||
| - | [[4f2g]] – OTC + phosphate – ''Burkholderia thailandensis''<br /> | ||
| - | [[4h31]] – VvOTC + norvaline + CP<br /> | ||
| - | [[4jfr]] – VvOTC + CP<br /> | ||
| - | [[4jhx]] – VvOTC + arginine + CP<br /> | ||
| - | [[4jqo]] – VvOTC + citrulline + phosphate<br /> | ||
| - | [[4nf2]] – BaOTC (mutant) + norvaline + CP<br /> | ||
| - | [[1c9y]] – hOTC + norvaline + carbamoyl phosphate - human<br /> | ||
| - | [[1ep9]], [[1fvo]] – hOTC + CP <br /> | ||
| - | [[1oth]] – hOTC + ornithine derivative<br /> | ||
| - | [[1duv]], [[2otc]] – EcOTC + ornithine derivative<br /> | ||
| - | [[1fb5]] – sOTC + norvaline - sheep<br /> | ||
== References == | == References == | ||
<references/> | <references/> | ||
[[Category:Topic Page]] | [[Category:Topic Page]] | ||
Current revision
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References
- ↑ Murata LB, Schachman HK. Structural similarity between ornithine and aspartate transcarbamoylases of Escherichia coli: characterization of the active site and evidence for an interdomain carboxy-terminal helix in ornithine transcarbamoylase. Protein Sci. 1996 Apr;5(4):709-18. PMID:8845761 doi:http://dx.doi.org/10.1002/pro.5560050416
- ↑ Storkanova G, Vlaskova H, Chuzhanova N, Zeman J, Stranecky V, Majer F, Peskova K, Luksan O, Jirsa M, Hrebicek M, Dvorakova L. Ornithine carbamoyltransferase deficiency: molecular characterization of 29 families. Clin Genet. 2013 Dec;84(6):552-9. doi: 10.1111/cge.12085. Epub 2013 Feb 7. PMID:23278509 doi:http://dx.doi.org/10.1111/cge.12085
- ↑ Sankaranarayanan R, Cherney MM, Cherney LT, Garen CR, Moradian F, James MN. The crystal structures of ornithine carbamoyltransferase from Mycobacterium tuberculosis and its ternary complex with carbamoyl phosphate and L-norvaline reveal the enzyme's catalytic mechanism. J Mol Biol. 2008 Jan 25;375(4):1052-63. Epub 2007 Nov 19. PMID:18062991 doi:10.1016/j.jmb.2007.11.025
