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Therapeutic concepts in succinate semialdehyde dehydrogenase (SSADH; ALDH5a1) deficiency (gamma-hydroxybutyric aciduria). Hypotheses evolved from 25 years of patient evaluation, studies in Aldh5a1-/- mice and characterization of gamma-hydroxybutyric acid pharmacology (English)

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author name string

I Knerr

1

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P L Pearl

2

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T Bottiglieri

3

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O Carter Snead

4

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C Jakobs

5

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K M Gibson

6

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language of work or name

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publication date

June 2007

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Journal of Inherited Metabolic Disease

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30

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3

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279-94

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Acamprosate enhances N-methyl-D-apartate receptor-mediated neurotransmission but inhibits presynaptic GABA(B) receptors in nucleus accumbens neurons

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21 January 2018

Quality assessment of urinary organic acid analysis.

1 reference

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21 January 2018

Increased guanidino species in murine and human succinate semialdehyde dehydrogenase (SSADH) deficiency

1 reference

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21 January 2018

Glutamine in the mechanism of ammonia-induced astrocyte swelling

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21 January 2018

Inhibition of succinic semialdehyde dehydrogenase activity by alkenal products of lipid peroxidation

1 reference

https://api.crossref.org/works/10.1007%2FS10545-007-0574-2

21 January 2018

Oxidation of [U-14C]succinic semialdehyde in cultured human lymphoblasts: measurement of residual succinic semialdehyde dehydrogenase activity in 11 patients with 4-hydroxybutyric aciduria

1 reference

https://api.crossref.org/works/10.1007%2FS10545-007-0574-2

21 January 2018

Clinical spectrum of succinic semialdehyde dehydrogenase deficiency

1 reference

https://api.crossref.org/works/10.1007%2FS10545-007-0574-2

21 January 2018

Taurine modulates catalase, aldehyde dehydrogenase, and ethanol elimination rates in rat brain.

1 reference

https://api.crossref.org/works/10.1007%2FS10545-007-0574-2

21 January 2018

Chronic intragastric administration of gamma-butyrolactone produces physical dependence in baboons.

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

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Photosensitive absence epilepsy with myoclonias and heterozygosity for succinic semialdehyde dehydrogenase (SSADH) deficiency

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

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Nitric Oxide Can Differentially Modulate Striatal Neurotransmitter Concentrations via Soluble Guanylate Cyclase and Peroxynitrite Formation

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Therapeutic intervention in mice deficient for succinate semialdehyde dehydrogenase (gamma-hydroxybutyric aciduria)

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Metabolism of gamma-hydroxybutyrate to d-2-hydroxyglutarate in mammals: further evidence for d-2-hydroxyglutarate transhydrogenase

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The clinical phenotype of succinic semialdehyde dehydrogenase deficiency (4-hydroxybutyric aciduria): case reports of 23 new patients.

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

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inferred from PubMed ID database lookup

Interaction between uridine and GABA-mediated inhibitory transmission: studies in vivo and in vitro.

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Presynaptic modulation of spontaneous inhibitory postsynaptic currents by gamma-hydroxybutyrate in the substantia nigra pars compacta

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Protective effect of melatonin in a chronic experimental model of Parkinson's disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Excitatory GABA responses in embryonic and neonatal cortical slices demonstrated by gramicidin perforated-patch recordings and calcium imaging.

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

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inferred from PubMed ID database lookup

A study of the enzymatic reactions involved in the formation of 5-hydroxy-4-ketohexanoic acid and its isomer, 5-keto-4-hydroxyhexanoic acid

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Oxidative stress and dopamine deficiency in a genetic mouse model of Lesch-Nyhan disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Uridine receptor: discovery and its involvement in sleep mechanism

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Ambient GABA constrains the strength of GABAergic synapses at Cajal-Retzius cells in the developing visual cortex.

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Expression profiling reveals multiple myelin alterations in murine succinate semialdehyde dehydrogenase deficiency.

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Changing properties of GABA(A) receptor-mediated signaling during early neocortical development.

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

gamma-Aminobutyric acid, acting through gamma -aminobutyric acid type A receptors, inhibits the biosynthesis of neurosteroids in the frog hypothalamus.

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Taurine prevents the neurotoxicity of beta-amyloid and glutamate receptor agonists: activation of GABA receptors and possible implications for Alzheimer's disease and other neurological disorders

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Succinic semialdehyde dehydrogenase deficiency: an inborn error of gamma-aminobutyric acid metabolism

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

SGS742: the first GABA(B) receptor antagonist in clinical trials

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Clinical aspects of the disorders of GABA metabolism in children

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Treatment with and withdrawal from finasteride alter ethanol intake patterns in male C57BL/6J mice: potential role of endogenous neurosteroids?

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Pharmacological treatment of alcohol dependence: target symptoms and target mechanisms

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Opposite effects of GABAB receptor antagonists on absences and convulsive seizures.

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Urinary organic acids in succinic semialdehyde dehydrogenase deficiency: evidence of alpha-oxidation of 4-hydroxybutyric acid, interaction of succinic semialdehyde with pyruvate dehydrogenase and possible secondary inhibition of mitochondrial beta-o

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

SSADH variation in primates: intra- and interspecific data on a gene with a potential role in human cognitive functions

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Identification of the gene encoding hydroxyacid-oxoacid transhydrogenase, an enzyme that metabolizes 4-hydroxybutyrate

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Murine succinate semialdehyde dehydrogenase (SSADH) deficiency, a heritable disorder of GABA metabolism with epileptic phenotype

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Dextromethorphan ameliorates effects of neonatal hypoxia on brain morphology and seizure threshold in rats.

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

GABA(B) receptors as potential therapeutic targets

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Neurotransmitter transporters and their impact on the development of psychopharmacology

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The effects and effectiveness of gamma-hydroxybutyrate in patients with narcolepsy.

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Vigabatrin therapy in six patients with succinic semialdehyde dehydrogenase deficiency.

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Gamma-hydroxybutyrate increases tryptophan availability and potentiates serotonin turnover in rat brain.

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Properties of succinic semialdehyde dehydrogenase in cultured human lymphoblasts.

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Taurine rescues hippocampal long-term potentiation from ammonia-induced impairment.

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Effect of valproic acid on the urinary metabolic profile of a patient with succinic semialdehyde dehydrogenase deficiency

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

GABAB receptor antagonism abolishes the learning impairments in rats with chronic atypical absence seizures.

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Basic pharmacology of valproate: a review after 35 years of clinical use for the treatment of epilepsy

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Enzyme and receptor antagonists for preventing toxicity from the gamma-hydroxybutyric acid precursor 1,4-butanediol in CD-1 mice

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Diagnostic challenges in a severely delayed infant with hypersomnolence, failure to thrive and arteriopathy: a unique case of gamma-hydroxybutyric aciduria and Williams syndrome.

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Selective gamma-hydroxybutyric acid receptor ligands increase extracellular glutamate in the hippocampus, but fail to activate G protein and to produce the sedative/hypnotic effect of gamma-hydroxybutyric acid.

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The Xyrem risk management program

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Clinical, cytogenetic and molecular characterization of a patient with combined succinic semialdehyde dehydrogenase deficiency and incomplete WAGR syndrome with obesity.

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The onset of brain injury and neurodegeneration triggers the synthesis of docosanoid neuroprotective signaling

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Taurine elevates dopamine levels in the rat nucleus accumbens; antagonism by strychnine

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Increased striatal dopamine synthesis is associated with decreased tissue levels of tyrosine

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Succinic semialdehyde dehydrogenase deficiency associated with combined 4-hydroxybutyric and dicarboxylic acidurias: potential for clinical misdiagnosis based on urinary organic acid profiling

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Astroglial contribution to brain energy metabolism in humans revealed by 13C nuclear magnetic resonance spectroscopy: elucidation of the dominant pathway for neurotransmitter glutamate repletion and measurement of astrocytic oxidative metabolism

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Seizure evolution and amino acid imbalances in murine succinate semialdehyde dehydrogenase (SSADH) deficiency

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Sedation with 4-hydroxybutyric acid: a potential pitfall in the diagnosis of SSADH deficiency.

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Absence seizures in succinic semialdehyde dehydrogenase deficient mice: a model of juvenile absence epilepsy.

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Spontaneous and precipitated withdrawal after chronic intragastric administration of gamma-hydroxybutyrate (GHB) in baboons

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Kinetic characterization of human hydroxyacid-oxoacid transhydrogenase: relevance to D-2-hydroxyglutaric and gamma-hydroxybutyric acidurias

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Acetoacetate protects neuronal cells from oxidative glutamate toxicity

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Superoxide dismutase, glutathione peroxidase activities and the hydroperoxide concentration are modified in the hippocampus of epileptic rats

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Gamma-Hydroxybutyrate inhibits excitatory postsynaptic potentials in rat hippocampal slices

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

gamma-Hydroxybutyrate induces cyclic AMP-responsive element-binding protein phosphorylation in mouse hippocampus: an involvement of GABA(B) receptors and cAMP-dependent protein kinase activation.

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Binding characteristics of the gamma-hydroxybutyric acid receptor antagonist [(3)H](2E)-(5-hydroxy-5,7,8,9-tetrahydro-6H-benzo[a][7]annulen-6-ylidene) ethanoic acid in the rat brain

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Gamma-hydroxybutyrate, gamma-butyrolactone, and 1,4-butanediol: a case report and review of the literature

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Significant behavioral disturbances in succinic semialdehyde dehydrogenase (SSADH) deficiency (Gamma-Hydroxybutyric aciduria)

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Focal neurometabolic alterations in mice deficient for succinate semialdehyde dehydrogenase

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Gamma-hydroxybutyrate--a drug of abuse

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

An overview of ?-hydroxybutyrate catabolism: The role of the cytosolic NADP+-dependent oxidoreductase EC 1.1.1.19 and of a mitochondrial hydroxyacid-oxoacid transhydrogenase in the initial, rate-limiting step in this pathway

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Effects of uridine in models of epileptogenesis and seizures.

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Resuscitative effect of a gamma-aminobutyric acid B receptor antagonist on gamma-hydroxybutyric acid mortality in mice

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Gammahydroxybutyrate: an endogenous regulator of energy metabolism

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Seizures in a boy with succinic semialdehyde dehydrogenase deficiency treated with vigabatrin (gamma-vinyl-GABA)

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Succinic semialdehyde dehydrogenase deficiency: GABAB receptor-mediated function

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The role of GABAergic neuroactive steroids in ethanol action, tolerance and dependence

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Succinate semialdehyde dehydrogenase deficiency does not down-regulate gamma-hydroxybutyric acid binding sites in the mouse brain

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Gamma-hydroxybutyric acid: neurobiology and toxicology of a recreational drug

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Block of cloned human T-type calcium channels by succinimide antiepileptic drugs.

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Thalamic mediodorsal and intralaminar nuclear lesions disrupt the generation of experimentally induced generalized absence-like seizures in rats.

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Inherited disorders of neurotransmitters in children and adults

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Enzymatic and immunologic identification of succinic semialdehyde dehydrogenase in rat and human neural and nonneural tissues

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

A ketogenic diet as a potential novel therapeutic intervention in amyotrophic lateral sclerosis

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The ketogenic diet: from molecular mechanisms to clinical effects

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Alterations in cytochrome c oxidase activity and energy metabolites in response to kainic acid-induced status epilepticus

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

D-2-hydroxyglutaric aciduria in three patients with proven SSADH deficiency: genetic coincidence or a related biochemical epiphenomenon?

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Murine succinate semialdehyde dehydrogenase deficiency

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Pharmacologic rescue of lethal seizures in mice deficient in succinate semialdehyde dehydrogenase

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Urinary excretion of gamma-hydroxybutyric acid in a patient with neurological abnormalities. The probability of a new inborn error of metabolism

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Vigabatrin and newer interventions in succinic semialdehyde dehydrogenase deficiency

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Streptozotocin-induced diabetes differentially modifies haloperidol- and gamma-hydroxybutyric acid (GHB)-induced catalepsy

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Carrier-mediated transport of baclofen across monolayers of bovine brain endothelial cells in primary culture

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Efficacy of the ketogenic diet as a treatment option for epilepsy: meta-analysis

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Involvement of gamma-hydroxybutyrate (GHB) and GABA-B receptors in the acute behavioral effects of GHB in baboons

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Binding characteristics of gamma-hydroxybutyric acid as a weak but selective GABAB receptor agonist.

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Taurine and neural cell damage

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Crystal structure of mouse succinic semialdehyde reductase AKR7A5: structural basis for substrate specificity

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Neurodevelopmental pattern of succinic semialdehyde dehydrogenase deficiency (gamma-hydroxybutyric aciduria).

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Magnetic resonance spectroscopy of neurotransmitters in human brain.

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Status epilepticus in mice deficient for succinate semialdehyde dehydrogenase: GABAA receptor-mediated mechanisms

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Succinic semialdehyde dehydrogenase deficiency (SSADH) (4-hydroxybutyric aciduria, gamma-hydroxybutyric aciduria).

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Substrate specificity of mouse aldo-keto reductase AKR7A5.

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Dextromethorphan alters gene expression in rat brain hippocampus and cortex.

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Thiamin-dependent enzymes as catalysts in chemoenzymatic syntheses.

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Mutational spectrum of the succinate semialdehyde dehydrogenase (ALDH5A1) gene and functional analysis of 27 novel disease-causing mutations in patients with SSADH deficiency

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Gamma-hydroxybutyrate is a weak agonist at recombinant GABA(B) receptors.

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Stable isotope dilution analysis of 4-hydroxybutyric acid: an accurate method for quantification in physiological fluids and the prenatal diagnosis of 4-hydroxybutyric aciduria

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Interactions of taurine and structurally related analogues with the GABAergic system and taurine binding sites of rabbit brain

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Presynaptic gamma-hydroxybutyric acid (GHB) and gamma-aminobutyric acidB (GABAB) receptor-mediated release of GABA and glutamate (GLU) in rat thalamic ventrobasal nucleus (VB): a possible mechanism for the generation of absence-like seizures [...]

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Vigabatrin therapy in patient with succinic semialdehyde dehydrogenase deficiency.

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Two exon-skipping mutations as the molecular basis of succinic semialdehyde dehydrogenase deficiency (4-hydroxybutyric aciduria)

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Metabolism of [U-14C]-4-hydroxybutyric acid to intermediates of the tricarboxylic acid cycle in extracts of rat liver and kidney mitochondria.

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Gamma-hydroxybutyric acid (GHB) and gamma-aminobutyric acidB receptor (GABABR) binding sites are distinctive from one another: molecular evidence.

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

4-Hydroxybutyric aciduria: application of a fluorometric assay to the determination of succinic semialdehyde dehydrogenase activity in extracts of cultured human lymphoblasts.

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Valproate: a reappraisal of its pharmacodynamic properties and mechanisms of action

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

L-2-Oxothiazolidine-4-carboxylic acid reverses endothelial dysfunction in patients with coronary artery disease.

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The peroxisome proliferator-activated receptor alpha-selective activator ciprofibrate upregulates expression of genes encoding fatty acid oxidation and ketogenesis enzymes in rat brain

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Low-carb diets, fasting and euphoria: Is there a link between ketosis and gamma-hydroxybutyrate (GHB)?

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Monitoring of 4‐hydroxybutyric acid levels in body fluids during vigabatrin treatment in succinic semialdehyde dehydrogenase deficiency

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Taurine interaction with neurotransmitter receptors in the CNS: an update.

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Isolation and characterization of a hydroxyacid-oxoacid transhydrogenase from rat kidney mitochondria

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Characterisation of a novel mouse liver aldo-keto reductase AKR7A5.

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The gamma-hydroxybutyrate signalling system in brain: organization and functional implications.

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Neuroprotective actions of selegiline

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Gamma-hydroxybutyrate reduces mitogen-activated protein kinase phosphorylation via GABA B receptor activation in mouse frontal cortex and hippocampus

1 reference

https://pubmed.ncbi.nlm.nih.gov/17457693

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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