Succinate semialdehyde dehydrogenase. Medical search. Frequent questions

In enzymology, a succinate-semialdehyde dehydrogenase (SSADH) (EC 1.2.1.24) is an enzyme that catalyzes the chemical reaction succinate semialdehyde + NAD+ + H2O ⇌ succinate + NADH + 2 H+ The 3 substrates of this enzyme are succinate semialdehyde, NAD+, and H2O, whereas its 3 products are succinate, NADH, and H+. (wikipedia.org)
Understanding the Molecular Mechanisms of Succinic Semialdehyde Dehydrogenase Deficiency (SSADHD): Towards the Development of SSADH-Targeted Medicine. (harvard.edu)
Mutational spectrum of the succinate semialdehyde dehydrogenase (ALDH5A1) gene and functional analysis of 27 novel disease-causing mutations in patients with SSADH deficiency. (medlineplus.gov)
Gordon N. Succinic semialdehyde dehydrogenase deficiency (SSADH) (4-hydroxybutyric aciduria, gamma-hydroxybutyric aciduria). (medlineplus.gov)
Succinic semialdehyde dehydrogenase (SSADH) deficiency is a rare inborn error of metabolism that is inherited in an autosomal recessive pattern. (rarediseases.org)
SSADH deficiency leads to abnormal accumulation of the compound succinic semialdehyde, which is reduced or converted to 4-hydroxybutyric acid, also known as GHB (gamma-hydroxybutyric acid). (rarediseases.org)
This report describes a case of severe lactic acidosis associated with a suspected succinic semialdehyde dehydrogenase (SSADH) deficiency in a 5-month-old Chihuahua. (huji.ac.il)

Variants (also called mutations) in the ALDH5A1 gene cause succinic semialdehyde dehydrogenase deficiency. (medlineplus.gov)
The ALDH5A1 gene provides instructions for producing the succinic semialdehyde dehydrogenase enzyme. (medlineplus.gov)
ALDH5A1 gene variants lead to a shortage (deficiency) of succinic semialdehyde dehydrogenase. (medlineplus.gov)

Bartsch, K., von Johnn-Marteville, A. and Schulz, A. Molecular analysis of two genes of the Escherichia coli gab cluster: nucleotide sequence of the glutamate:succinic semialdehyde transaminase gene ( gabT ) and characterization of the succinic semialdehyde dehydrogenase gene ( gabD ). (enzyme-database.org)

Other names in common use include succinate semialdehyde dehydrogenase, succinic semialdehyde dehydrogenase, succinyl semialdehyde dehydrogenase, and succinate semialdehyde:NAD+ oxidoreductase. (wikipedia.org)

The systematic name of this enzyme class is succinate-semialdehyde:NAD+ oxidoreductase. (wikipedia.org)

This gene encodes a mitochondrial NAD(+)-dependent succinic semialdehyde dehydrogenase. (nih.gov)

Probable methylmalonate-semialdehyde dehydrogenase MmsA. (ntu.edu.sg)

The scientists discovered that Synechococcus indeed had genes that coded for one important alternative enzyme, succinic semialdehyde dehydrogenase, and that adjacent to the gene for this enzyme was a misidentified gene that subsequently was shown to encode a novel enzyme, 2-oxo-glutarate decarboxylase. (world.edu)

It is important in the degradation of γ-aminobutyric acid in humans, and deficiency of the enzyme causes serious health effects (succinic semialdehyde dehydrogenase deficiency). (wikipedia.org)
Establishment and validation of a clinical severity scoring system for succinic semialdehyde dehydrogenase deficiency. (harvard.edu)
Development of a Quality-of-Life Survey for Patients With Succinic Semialdehyde Dehydrogenase Deficiency, a Rare Disorder of GABA Metabolism. (harvard.edu)
Transcranial Magnetic Stimulation in Succinic Semialdehyde Dehydrogenase Deficiency: A Measure of Maturational Trajectory of Cortical Excitability. (harvard.edu)
aminobutyric acid B (GABA-B) Receptor Antagonist, for Succinic Semialdehyde Dehydrogenase Deficiency. (harvard.edu)
Speech Motor Function and Auditory Perception in Succinic Semialdehyde Dehydrogenase Deficiency: Toward Pre-Supplementary Motor Area (SMA) and SMA-Proper Dysfunctions. (harvard.edu)
Postmortem Analyses in a Patient With Succinic Semialdehyde Dehydrogenase Deficiency (SSADHD): II. (harvard.edu)
Magnetic Resonance Imaging (MRI) and Spectroscopy in Succinic Semialdehyde Dehydrogenase Deficiency. (harvard.edu)
Succinic semialdehyde dehydrogenase deficiency is a disorder that can cause a variety of neurological problems. (medlineplus.gov)
About half of people with succinic semialdehyde dehydrogenase deficiency experience seizures, difficulty coordinating movements (ataxia), decreased reflexes (hyporeflexia), and behavioral problems. (medlineplus.gov)
Approximately 350 people with succinic semialdehyde dehydrogenase deficiency have been reported worldwide. (medlineplus.gov)
It is unclear how an increase in GABA and GHB causes developmental delays, seizures, and other signs and symptoms of succinic semialdehyde dehydrogenase deficiency. (medlineplus.gov)
Succinic Semialdehyde Dehydrogenase Deficiency: Review of the Natural History Study. (medlineplus.gov)
Succinic semialdehyde dehydrogenase deficiency (SSADHD), also known as 4-hydoxybutyric aciduria or gamma-hydoxybutyric aciduria , is a rare autosomal recessive disorder [1] of the degradation pathway of the inhibitory neurotransmitter γ-aminobutyric acid , or GABA . (wikidoc.org)
However, because of the deficiency, the final intermediate of the GABA degradation pathway, succinic semialdehyde, accumulates and cannot be oxidized to succinic acid and is therefore reduced to gamma-hydroxybutyric acid (GHB) by gamma-hydroxybutyric dehydrogenase. (wikidoc.org)
Succinic semialdehyde dehydrogenase deficiency has an autosomal recessive pattern of inheritance. (wikidoc.org)

Catalyzes the NADPH-dependent reduction of succinyl-CoA to succinate semialdehyde. (expasy.org)
Comment: This may be either NADH or NADPH dependent, forming succinate in either case. (lbl.gov)

Horváth G, Sváb G, Komlódi T, Ravasz D, Kacsó G, Doczi J, Chinopoulos C, Ambrus A, Tretter L (2022) Reverse and forward electron flow-induced H 2 O 2 formation is decreased in α-ketoglutarate dehydrogenase (α-KGDH) subunit (E2 or E3) heterozygote knock out animals. (mitophysiology.org)

An enzyme that plays a role in the GLUTAMATE and butanoate metabolism pathways by catalyzing the oxidation of succinate semialdehyde to SUCCINATE using NAD+ as a coenzyme. (harvard.edu)

That combination of enzymes allows these organisms to move to the next intermediate - succinate - and to complete the TCA cycle. (world.edu)

That is, rather than making 2-oxoglutarate dehydrogenase, these bacteria produce both 2-oxoglutarate decarboxylase and succinic semialdehyde dehydrogenase. (world.edu)

Retinal dehydrogenase. (uma.es)

In bacteria, the enzyme is also involved in γ-aminobutyric acid degradation, but can be recruited to facilitate other functions, such as converting succinate-semialdehyde formed during fission of the pyridine ring to succinic acid for entry into the Krebs Cycle. (wikipedia.org)
GABA is metabolized by successive transamination and oxidation to yield succinic semialdehyde and succinic acid respectively via the catalyzing effects of GABA transaminase . (wikidoc.org)

This protein belongs to the aldehyde dehydrogenase family of proteins. (nih.gov)

The enzyme has been described in Clostridium kluyveri, where it participates in succinate fermentation, and in Metallosphaera sedula, where it participates in the 3-hydroxypropanoate/4-hydroxybutyrate cycle, an autotrophic CO2 fixation pathway found in some thermoacidophilic archaea. (expasy.org)

Jaeger, M., Rothacker, B. and Ilg, T. Saturation transfer difference NMR studies on substrates and inhibitors of succinic semialdehyde dehydrogenases. (enzyme-database.org)

Aldehyde dehydrogenase family [Interproscan]. (ntu.edu.sg)

Succinate-semialdehyde dehydrogenase is found in organisms ranging across the tree of life from bacteria to humans. (wikipedia.org)
They concluded that cyanobacteria lacked the ability to make one enzyme, called 2-oxoglutarate dehydrogenase, and that this missing enzyme rendered the bacteria unable to produce a compound - called succinyl-coenzyme A - for the next step in the TCA cycle. (world.edu)

putative NAD-dependent aldehyde dehydrogenase [Ensembl]. (ntu.edu.sg)

This graph shows the total number of publications written about "Succinate-Semialdehyde Dehydrogenase" by people in Harvard Catalyst Profiles by year, and whether "Succinate-Semialdehyde Dehydrogenase" was a major or minor topic of these publication. (harvard.edu)
Below are the most recent publications written about "Succinate-Semialdehyde Dehydrogenase" by people in Profiles. (harvard.edu)

Conversely, cytokinin dehydrogenase 6 ( CKX6 ) was found to be up-regulated in male flowers of T. fortunei , suggesting that a lower concentration of CK is more conducive to male flower development [ 6 ]. (beds.ac.uk)

It is important in the degradation of γ-aminobutyric acid in humans, and deficiency of the enzyme causes serious health effects (succinic semialdehyde dehydrogenase deficiency). (wikipedia.org)
Succinic semialdehyde dehydrogenase deficiency is a disorder that can cause a variety of neurological problems. (medlineplus.gov)
Less common features of succinic semialdehyde dehydrogenase deficiency include uncontrollable movements of the limbs (choreoathetosis), involuntary tensing of the muscles (dystonia), muscle twitches (myoclonus), and a progressive worsening of ataxia. (medlineplus.gov)
Approximately 350 people with succinic semialdehyde dehydrogenase deficiency have been reported worldwide. (medlineplus.gov)
Mutations in the ALDH5A1 gene cause succinic semialdehyde dehydrogenase deficiency. (medlineplus.gov)
It is unclear how an increase in GABA and GHB causes developmental delay, seizures, and other signs and symptoms of succinic semialdehyde dehydrogenase deficiency. (medlineplus.gov)
Gordon N. Succinic semialdehyde dehydrogenase deficiency (SSADH) (4-hydroxybutyric aciduria, gamma-hydroxybutyric aciduria). (medlineplus.gov)
His laboratory employs pharmacological, cellular and dietary treatment approaches in disorders such as succinic semialdehyde dehydrogenase deficiency, phenylketonuria, maple syrup urine disease, galactosemia and transaldolase deficiency, a defect of the pentose phosphate pathway. (wsu.edu)
Gibson is the chair of the Medical and Scientific Advisory Board of the SSADH Association , which represents children and families affected by Succinic Semialdehyde Dehydrogenase Deficiency (SSADH). (wsu.edu)

This gene encodes a mitochondrial NAD(+)-dependent succinic semialdehyde dehydrogenase. (novusbio.com)
Our study implies that bryophytes but presumably also some green algae, lycopods and ferns, which carry both ALDH21 and ALDH5 genes, can oxidize SSAL to succinate in both cytosol and mitochondria, indicating a more diverse GABA shunt pathway compared with higher plants carrying only the mitochondrial ALDH5. (rcsb.org)
10. Higher Vulnerability of Menadione-Exposed Cortical Astrocytes of Glutaryl-CoA Dehydrogenase Deficient Mice to Oxidative Stress, Mitochondrial Dysfunction, and Cell Death: Implications for the Neurodegeneration in Glutaric Aciduria Type I. (nih.gov)
12. Interaction of glutaric aciduria type 1-related glutaryl-CoA dehydrogenase with mitochondrial matrix proteins. (nih.gov)

A shortage (deficiency) of succinic semialdehyde dehydrogenase leads to an increase in the amount of GABA and a related molecule called gamma-hydroxybutyrate (GHB) in the body, particularly the brain and spinal cord (central nervous system). (medlineplus.gov)
We constructed vectors expressing ScGABA-TKG and AtGABA-TP in both yeast cytosol and mitochondria to complement yeast GABA transaminase mutant ?uga1 and succinate semialdehyde dehydrogenase mutant ?uga2 phenotypes: GABA growth defect, thermo sensitivity and heat induced production of reactive oxygen species (ROS). (auburn.edu)
Here, we show that ALDH21 from the moss Physcomitrella patens codes for a tetrameric NADP + -dependent succinic semialdehyde dehydrogenase (SSALDH), which converts succinic semialdehyde, an intermediate of the γ-aminobutyric acid (GABA) shunt pathway, into succinate in the cytosol. (rcsb.org)
This study aimed to determine the effects of changes of faecal microbiota in obese and diabetic rats on the imputed production of enzymes involved in the metabolism of glutamate, gamma-aminobutyric acid (GABA), and succinate. (edu.krd)
The levels of glutamate decarboxylase, GABA transaminase, succinate-semialdehyde dehydrogenase, and methylisocitrate lyase were reduced or absent in diabetic rats compared with controls and obese rats. (edu.krd)
These findings suggest that the absence of GABA and reduced succinate metabolism from gut microbiota contribute to the diabetic state in rats. (edu.krd)
GABA transaminase is a key enzyme that converts GABA to succinate semialdehyde (SSA). (chinacrops.org)
It also operates a cyclic TCA cycle via the γ-aminobutyric acid (GABA) shunt, which forms succinate through the intermediates glutamate and succinate semialdehyde, despite the missing enzyme from 2-oxoglutarate to succinyl-CoA [ 7 ]. (biomedcentral.com)

Saccharopine dehydrogenase C-terminal domain, Saccharopine dehydrogenase NADP binding domain [Interproscan]. (ntu.edu.sg)
Glycerol kinase and NADP + -dependent glycerol dehydrogenase activities were present under all physiological conditions tested. (microbiologyresearch.org)

A functional polymorphism in the succinate-semialdehyde dehydrogenase (aldehyde dehydrogenase 5 family, member A1) gene is associated with cognitive ability. (cdc.gov)

Aldehyde dehydrogenase (ALDH) converts aldehydes to carboxylic acids while reducing NAD+ to NADH. (proteopedia.org)

15. Glutaric aciduria type 1 metabolites impair the succinate transport from astrocytic to neuronal cells. (nih.gov)

Succinate supplements have been shown to help reduce the effects of hangovers by activating the degradation of acetaldehyde - a toxic byproduct of alcohol metabolism - into CO2 and H2O through aerobic metabolism. (drugbank.com)
An enzyme that plays a role in the GLUTAMATE and butanoate metabolism pathways by catalyzing the oxidation of succinate semialdehyde to SUCCINATE using NAD + as a coenzyme. (nih.gov)
7. Oxidative Stress, Disrupted Energy Metabolism, and Altered Signaling Pathways in Glutaryl-CoA Dehydrogenase Knockout Mice: Potential Implications of Quinolinic Acid Toxicity in the Neuropathology of Glutaric Acidemia Type I. (nih.gov)

RALDH class 1A 1 is retinal dehydrogenase which converts retinalaldehyde to retinoic acid. (proteopedia.org)
RALDH class 1A 2 is retinal dehydrogenase which converts retinal to retinoate. (proteopedia.org)

use ACYLTRANSFERASES 1973-1979, use COENZYME A & PHOSPHOLIPIDS 1973-1978 MH - 1-Pyrroline-5-Carboxylate Dehydrogenase UI - D050842 MN - D8.811.682.662.693 MS - An enzyme that catalyzes the oxidation of 1-pyrroline-5-carboxylate to L-GLUTAMATE in the presence of NAD. (nih.gov)
HN - 2006(1983) MH - 2-Oxoisovalerate Dehydrogenase (Acylating) UI - D050645 MN - D8.811.682.657.350.825 MS - An NAD+ dependent enzyme that catalyzes the oxidation 3-methyl-2-oxobutanoate to 2-methylpropanoyl-CoA. (nih.gov)
use AMINO ACIDS, BRANCHED-CHAIN 1979, & KETO ACIDS & VALERATES 1973-1979 MH - 3-Hydroxyanthranilate 3,4-Dioxygenase UI - D050561 MN - D8.811.682.690.416.328 MS - An enzyme that catalyzes the conversion of 3-hydroxyanthranilate to 2-amino-3-carboxymuconate semialdehyde. (nih.gov)
use ANTHRANILIC ACID 1974-1979 MH - 3-Isopropylmalate Dehydrogenase UI - D050539 MN - D8.811.682.47.500 MS - An NAD+ dependent enzyme that catalyzes the oxidation of 3-carboxy-2-hydroxy-4-methylpentanoate to 3-carboxy-4-methyl-2-oxopentanoate. (nih.gov)

We also show that pyruvate decarboxylase (AceE), the E1 component of pyruvate dehydrogenase (PDHC), can participate in AceE/DlaT/AhpD/AhpC, using pyruvate as a source of electrons for reductase action. (cornell.edu)

The recombinant protein was highly specific for succinate semialdehyde, can use both NAD+ and NADP+ as a cofactor but with higher affinity to NAD+. (auburn.edu)

RALDH class 1A 3 is retinal dehydrogenase which funcrions in detoxification of aldehydes. (proteopedia.org)
In the former role, HOAS acts to prevent accumulation of aldehydes, including growth-inhibitory succinate semialdehyde (SSA). (cornell.edu)

These three enzymes acting in concert convert glutamate to succinate. (auburn.edu)

2014). A gatekeeper helix determines the substrate specificity of Sjögren-Larsson Syndrome enzyme fatty aldehyde dehydrogenase. (proteopedia.org)

In bacteria, the enzyme is also involved in γ-aminobutyric acid degradation, but can be recruited to facilitate other functions, such as converting succinate-semialdehyde formed during fission of the pyridine ring to succinic acid for entry into the Krebs Cycle. (wikipedia.org)

Succinate is an essential component of the Krebs or citric acid cycle and serves an electron donor in the production of fumaric acid and FADH2. (drugbank.com)

The crystal structure with the bound product succinate shows that its carboxylate group establishes salt bridges with both Arg-121 and Arg-457, and a hydrogen bond with Tyr-296. (rcsb.org)

SSADH7

ALDH5A13

GabD1

Succinyl semialdehyde dehydrogenase1

Oxidoreductase1

Mitochondrial1

Methylmalonate-semialdehyde1

Gene1

Deficiency16

NADPH2

Subunit1

Glutamate1

Enzymes1

Decarboxylase1

Retinal1

Succinic acid2

Protein1

Participates1

Substrates1

Interproscan1

Bacteria2

Putative1

Profiles2

Found1

Succinic semialdehy9

Mitochondrial4

GABA8

NADP2

Aldehyde dehydrogenase 5 family1

NADH1

Aciduria1

Metabolism3

Converts2

Enzyme that catalyzes4

Pyruvate1

Protein1

Aldehydes2

Enzymes1

Substrate1

Entry1

Component1

Product1