Succinate-semialdehyde dehydrogenase: Difference between revisions

Revision as of 15:37, 4 September 2016

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^[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 substrate succinate semialdehyde and the cofactor NADP^[3].

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

Drag the structure with the mouse to rotate

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

Updated on 04-September-2016

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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	<StructureSection load='3vz3' size='~~340~~' side='right' caption='Structure of succinate-semialdehyde dehydrogenase complex with NADPH and succinate semialdehyde (PDB code [[3vz3]]).' scene=''>		<StructureSection load='3vz3' size='450' side='right' caption='Structure of succinate-semialdehyde dehydrogenase complex with NADPH and succinate semialdehyde (PDB code [[3vz3]]).' scene='59/590825/Cv/2'>

	== Function ==		== Function ==