Succinate-semialdehyde dehydrogenase: Difference between revisions

Revision as of 15:09, 16 March 2021

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]. 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 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 16-March-2021

ReferencesReferences

↑ 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:http://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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Michal Harel, Alexander Berchansky, Joel L. Sussman

[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

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 == 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<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>. See also [[Aldehyde dehydrogenase]].
 == Disease ==