Succinate-semialdehyde dehydrogenase: Difference between revisions

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== Function ==
== Function ==


'''Succinate-semialdehyde dehydrogenase''' (SSD) catalyzes the conversion of succinate semialdehyde, NAD+ and water to succinate, NADH and H+.  SSD participates in glutamate and butyrate metabolism<ref>PMID:13654295</ref>.
'''Succinate-semialdehyde dehydrogenase''' or '''α-ketoglutaric semialdehyde dehydrogenase''' (SSD) catalyzes the conversion of succinate semialdehyde, NAD+ and water to succinate, NADH and H+.  SSD participates in glutamate and butyrate metabolism<ref>PMID:13654295</ref>.


== Disease ==
== Disease ==
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*Succinate-semialdehyde dehydrogenase
*Succinate-semialdehyde dehydrogenase


**[[2w8n]], [[2w8o]] – hSSD - human <br />
**[[2w8p]] – hSSD (mutant) <br />
**[[4it9]], [[3vz1]] – SySSD - ''Synechococcus''<br />
**[[4it9]], [[3vz1]] – SySSD - ''Synechococcus''<br />
**[[3vz2]] – SySSD (mutant) <br />
**[[3vz2]] – SySSD (mutant) <br />
Line 26: Line 28:
**[[5vbf]] – SSD – ''Burkholderia vietnamiensis''<br />
**[[5vbf]] – SSD – ''Burkholderia vietnamiensis''<br />
**[[3rh9]] – SSD – ''Marinobacter aquaeolei''<br />
**[[3rh9]] – SSD – ''Marinobacter aquaeolei''<br />
**[[2w8n]], [[2w8o]] – hSSD - human <br />
**[[2w8p]] – hSSD (mutant) <br />
**[[4ogd]] – SpSSD – ''Streptococcus pyogenes'' <br />
**[[4ogd]] – SpSSD – ''Streptococcus pyogenes'' <br />
**[[5x5t]] – AbSSD – ''Azuspirillum brasilense'' <br />


*Succinate-semialdehyde dehydrogenase complexes
*Succinate-semialdehyde dehydrogenase complexes
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**[[4oht]] – SpSSD + NADPH<br />
**[[4oht]] – SpSSD + NADPH<br />
**[[4ywu]], [[4ywv]] – SySSD + oxobutanoic acid + succinic semialdehyde<br />
**[[4ywu]], [[4ywv]] – SySSD + oxobutanoic acid + succinic semialdehyde<br />
**[[5x5u]] – AbSSD + NAD<br />


}}
}}

Revision as of 11:08, 11 February 2020


Function

Succinate-semialdehyde dehydrogenase or α-ketoglutaric semialdehyde dehydrogenase (SSD) catalyzes the conversion of succinate semialdehyde, NAD+ and water to succinate, NADH and H+. SSD participates in glutamate and butyrate metabolism[1].

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 and the [3]. Water molecules are shown as red spheres. .


Structure of succinate-semialdehyde dehydrogenase complex with NADPH and succinate semialdehyde (PDB code 3vz3).

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3D structures of succinate-semialdehyde dehydrogenase3D structures of succinate-semialdehyde dehydrogenase

Updated on 11-February-2020

ReferencesReferences

  1. JAKOBY WB, SCOTT EM. Aldehyde oxidation. III. Succinic semialdehyde dehydrogenase. J Biol Chem. 1959 Apr;234(4):937-40. PMID:13654295
  2. 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:http://dx.doi.org/10.1016/j.ejpn.2004.06.004
  3. 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

Proteopedia Page Contributors and Editors (what is this?)Proteopedia Page Contributors and Editors (what is this?)

Michal Harel, Alexander Berchansky, Joel L. Sussman