Cystathionine beta-synthase
From Proteopedia
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<StructureSection load='4pcu' size='340' side='right' caption='Human cystathionine β-synthase complex with heme, PLP and SAM (PDB id [[4pcu]]) ' scene=''> | <StructureSection load='4pcu' size='340' side='right' caption='Human cystathionine β-synthase complex with heme, PLP and SAM (PDB id [[4pcu]]) ' scene=''> | ||
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== Function == | == Function == | ||
| - | '''Cystathionine β-synthase''' (CBS) catalyzes the interconversion of cysteine and homocysteine. In bacterial systems CBS catalyzes the transfer of thiol from cysteine to homocysteine and in mammalian systems from homocysteine to cysteine. | + | '''Cystathionine β-synthase''' (CBS) catalyzes the PLP-dependent interconversion of cysteine and homocysteine. In bacterial systems CBS catalyzes the transfer of thiol from cysteine to homocysteine and in mammalian systems from homocysteine to cysteine<ref>PMID:32365821</ref>.. |
== Disease == | == Disease == | ||
| - | + | CBS deficiency causes homocysteinuria which affects the eye, skeletal system, vascular system and the CNS<ref>PMID:20301697</ref>. Mutations in Ala114, Ile278, G305 and G307 were found to be involved in homocysteinuria<ref>PMID:15087459</ref>. | |
== Structural highlights == | == Structural highlights == | ||
| - | + | The structure of the complex of CBS with heme, PLP and SAM shows the N-terminal domain which binds the heme cofactor interacting with Cys52 and His65. The catalytic core interacts with the PLP cofactor at Lys119 and a C-terminal regulatory domain. | |
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| + | ==3D structures of cystathionine β-synthase== | ||
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| + | [[Cystathionine β-synthase 3D structures]] | ||
</StructureSection> | </StructureSection> | ||
== References == | == References == | ||
<references/> | <references/> | ||
| + | [[Category:Topic Page]] | ||
Current revision
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References
- ↑ Zuhra K, Augsburger F, Majtan T, Szabo C. Cystathionine-beta-Synthase: Molecular Regulation and Pharmacological Inhibition. Biomolecules. 2020 Apr 30;10(5). pii: biom10050697. doi: 10.3390/biom10050697. PMID:32365821 doi:http://dx.doi.org/10.3390/biom10050697
- ↑ Picker JD, Levy HL. Homocystinuria Caused by Cystathionine Beta-Synthase Deficiency PMID:20301697
- ↑ Miles EW, Kraus JP. Cystathionine beta-synthase: structure, function, regulation, and location of homocystinuria-causing mutations. J Biol Chem. 2004 Jul 16;279(29):29871-4. Epub 2004 Apr 15. PMID:15087459 doi:http://dx.doi.org/10.1074/jbc.R400005200
