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Function
Succinate-semialdehyde dehydrogenase or α-ketoglutaric semialdehyde dehydrogenase or succinyl-CoA reductase (SSD) catalyzes the conversion of succinate semialdehyde, NAD+ and water to succinate, NADH and H+. SSD participates in glutamate and butyrate metabolism[1]. See also Aldehyde dehydrogenase.
Disease
SSD deficiency is a disorder of GABA metabolism with symptoms of seizures, delayed development and hypotonia[2].
Structural highlights
The active site of SSD contains the substrate succinate semialdehyde and the cofactor NADP[3]. Water molecules are shown as red spheres. Whole binding site.
- ↑ JAKOBY WB, SCOTT EM. Aldehyde oxidation. III. Succinic semialdehyde dehydrogenase. J Biol Chem. 1959 Apr;234(4):937-40. PMID:13654295
- ↑ Gordon N. Succinic semialdehyde dehydrogenase deficiency (SSADH) (4-hydroxybutyric aciduria, gamma-hydroxybutyric aciduria). Eur J Paediatr Neurol. 2004;8(5):261-5. PMID:15341910 doi:https://dx.doi.org/10.1016/j.ejpn.2004.06.004
- ↑ Yuan Z, Yin B, Wei D, Yuan YR. Structural basis for cofactor and substrate selection by cyanobacterium succinic semialdehyde dehydrogenase. J Struct Biol. 2013 May;182(2):125-35. doi: 10.1016/j.jsb.2013.03.001. Epub 2013 , Mar 13. PMID:23500184 doi:10.1016/j.jsb.2013.03.001
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3D structures of succinate-semialdehyde dehydrogenase
Updated on 06-December-2023
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- Succinate-semialdehyde dehydrogenase
- Aldehyde dehydrogenase, 2w8o – hSSD - human
- 2w8p – hSSD (mutant)
- 4it9, 3vz1 – SySSD - Synechococcus
- 3vz2 – SySSD (mutant)
- 4v6h – SSD – Burkholderia pseudomallei
- 5vbf – SSD – Burkholderia vietnamiensis
- 3rh9 – SSD – Marinobacter aquaeolei
- 4ogd – SpSSD – Streptococcus pyogenes
- 5x5t – AbSSD – Azuspirillum brasilense
- Succinate-semialdehyde dehydrogenase complexes
- 2w8q – hSSD (mutant) + succinate
- 2w8r – hSSD (mutant) + ADP
- 4ita – SySSD + NADPH
- 4itb – SySSD + NADPH + succinic semialdehyde
- 3vz3 – SySSD (mutant) + NADPH + succinic semialdehyde
- 3jz4 – SSD + NADPH – Escherichia coli
- 4oht – SpSSD + NADPH
- 4ywu, 4ywv – SySSD + oxobutanoic acid + succinic semialdehyde
- 5x5u – AbSSD + NAD
- 8c54 – SSD + NADH – Rhizobium leguminosarum – Cryo EM
- 8cej – CkSSD + mesaconyl-c1-CoA – Clostridium kluyveri
- 8cek – CkSSD + NADPH
References
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