Succinic semialdehyde dehydrogenase: biochemical-molecular-clinical disease mechanisms, redox regulation, and functional significance (Q28743137)

2

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Kimmo Jensen

3

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O Carter Snead

4

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Patrizia Malaspina

5

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Cornelis Jakobs

6

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K Michael Gibson

Antioxidants & Redox Signaling

7

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language of work or name

English

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publication date

1 August 2011

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published in

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volume

15

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issue

3

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page(s)

691-718

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cites work

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A ketogenic diet rescues the murine succinic semialdehyde dehydrogenase deficient phenotype

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Altered cerebral glucose and acetate metabolism in succinic semialdehyde dehydrogenase-deficient mice: evidence for glial dysfunction and reduced glutamate/glutamine cycling

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Enzymatic and metabolic evidence for a region specific mitochondrial dysfunction in brains of murine succinic semialdehyde dehydrogenase deficiency (Aldh5a1-/- mice)

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Lipid abnormalities in succinate semialdehyde dehydrogenase (Aldh5a1-/-) deficient mouse brain provide additional evidence for myelin alterations

16 March 2017

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Crystal Structure of Lactaldehyde Dehydrogenase from Escherichia coli and Inferences Regarding Substrate and Cofactor Specificity

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Succinic semialdehyde dehydrogenase deficiency: GABAB receptor-mediated function

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

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

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GABAB receptors in Schwann cells influence proliferation and myelin protein expression

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Proton MR spectroscopy in succinic semialdehyde dehydrogenase deficiency

16 March 2017

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Clinical aspects of the disorders of GABA metabolism in children

16 March 2017

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Succinic semialdehyde dehydrogenase deficiency in children and adults

16 March 2017

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Vigabatrin and newer interventions in succinic semialdehyde dehydrogenase deficiency

16 March 2017

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Oxidation of 4-hydroxy-2-nonenal by succinic semialdehyde dehydrogenase (ALDH5A)

16 March 2017

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

16 March 2017

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Focal neurometabolic alterations in mice deficient for succinate semialdehyde dehydrogenase

16 March 2017

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

16 March 2017

1 reference

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

16 March 2017

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Structural basis of the redox switch in the OxyR transcription factor

16 March 2017

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Sheep liver cytosolic aldehyde dehydrogenase: the structure reveals the basis for the retinal specificity of class 1 aldehyde dehydrogenases

16 March 2017

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

16 March 2017

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4-Hydroxybutyric acid and the clinical phenotype of succinic semialdehyde dehydrogenase deficiency, an inborn error of GABA metabolism

16 March 2017

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Structure of mitochondrial aldehyde dehydrogenase: the genetic component of ethanol aversion

16 March 2017

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Human succinic semialdehyde dehydrogenase. Molecular cloning and chromosomal localization

16 March 2017

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Inherited disorders of GABA metabolism

16 March 2017

1 reference

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Molecular cloning of the mature NAD(+)-dependent succinic semialdehyde dehydrogenase from rat and human. cDNA isolation, evolutionary homology, and tissue expression

16 March 2017

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Kinetic Studies with Rat‐Brain Succinic‐Semialdehyde Dehydrogenase

16 March 2017

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

16 March 2017

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

16 March 2017

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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)

16 March 2017

1 reference

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Variations on an inhibitory theme: phasic and tonic activation of GABA(A) receptors

16 March 2017

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GABAergic control of neurite outgrowth and remodeling during development and adult neurogenesis: general rules and differences in diverse systems.

7 April 2017

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Effect of gene dosage on single-cell hippocampal electrophysiology in a murine model of SSADH deficiency (gamma-hydroxybutyric aciduria).

3 June 2018

1 reference

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Neuropathology in succinic semialdehyde dehydrogenase deficiency

3 June 2018

1 reference

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Corticotropin-releasing hormone, glutamate, and γ-aminobutyric acid in depression

3 June 2018

1 reference

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Regulation of GABA(A) receptor subunit expression by pharmacological agents.

3 June 2018

1 reference

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Polysomnographic abnormalities in succinic semialdehyde dehydrogenase (SSADH) deficiency

3 June 2018

1 reference

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Neuronal and non-neuronal GABA transporters as targets for antiepileptic drugs

3 June 2018

1 reference

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Evidence for genetic heterogeneity in D-2-hydroxyglutaric aciduria

3 June 2018

1 reference

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Neuronal glutamate and GABAA receptor function in health and disease

3 June 2018

1 reference

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Developing analgesics by enhancing spinal inhibition after injury: GABAA receptor subtypes as novel targets

3 June 2018

1 reference

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Decreased GABA-A binding on FMZ-PET in succinic semialdehyde dehydrogenase deficiency.

3 June 2018

1 reference

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Narcolepsy and depression and the neurobiology of gammahydroxybutyrate

3 June 2018

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GABA(A) receptors and their associated proteins: implications in the etiology and treatment of schizophrenia and related disorders

3 June 2018

1 reference

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Visual evoked potentials in succinate semialdehyde dehydrogenase (SSADH) deficiency

3 June 2018

1 reference

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Behavioral effects and pharmacokinetics of gamma-hydroxybutyrate (GHB) precursors gamma-butyrolactone (GBL) and 1,4-butanediol (1,4-BD) in baboons

3 June 2018

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Succinic semialdehyde dehydrogenase deficiency: lessons from mice and men

3 June 2018

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The effects of a ketogenic diet on ATP concentrations and the number of hippocampal mitochondria in Aldh5a1(-/-) mice

3 June 2018

1 reference

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Comparative genomics of aldehyde dehydrogenase 5a1 (succinate semialdehyde dehydrogenase) and accumulation of gamma-hydroxybutyrate associated with its deficiency

3 June 2018

1 reference

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Neurotransmitter alterations in embryonic succinate semialdehyde dehydrogenase (SSADH) deficiency suggest a heightened excitatory state during development

3 June 2018

1 reference

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Lipid peroxidation of membrane phospholipids generates hydroxy-alkenals and oxidized phospholipids active in physiological and/or pathological conditions

3 June 2018

1 reference

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GABA, a natural immunomodulator of T lymphocytes

3 June 2018

1 reference

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The redox environment in the mitochondrial intermembrane space is maintained separately from the cytosol and matrix

3 June 2018

1 reference

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SSADH deficiency leads to elevated extracellular GABA levels and increased GABAergic neurotransmission in the mouse cerebral cortex.

3 June 2018

1 reference

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Neuropsychiatric morbidity in adolescent and adult succinic semialdehyde dehydrogenase deficiency patients

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Circadian distribution of generalized tonic-clonic seizures associated with murine succinic semialdehyde dehydrogenase deficiency, a disorder of GABA metabolism.

3 June 2018

1 reference

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Dynamics driving function: new insights from electron transferring flavoproteins and partner complexes.

3 June 2018

1 reference

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The redox-switch domain of Hsp33 functions as dual stress sensor

3 June 2018

1 reference

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The PAH gene, phenylketonuria, and a paradigm shift

3 June 2018

1 reference

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Aldehyde metabolism in the cardiovascular system.

3 June 2018

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Gamma-hydroxybutyric acid induces oxidative stress in cerebral cortex of young rats

3 June 2018

1 reference

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Targets for antiepileptic drugs in the synapse

3 June 2018

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Protein adducts generated from products of lipid oxidation: focus on HNE and one.

3 June 2018

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GABA receptor-mediated effects in the peripheral nervous system: A cross-interaction with neuroactive steroids.

3 June 2018

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

3 June 2018

1 reference

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GABA-based therapeutic approaches: GABAA receptor subtype functions

3 June 2018

1 reference

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Inherited disorders of neurotransmitters in children and adults

3 June 2018

1 reference

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

3 June 2018

1 reference

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Analysis and update of the human aldehyde dehydrogenase (ALDH) gene family

3 June 2018

1 reference

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Gamma-hydroxybutyric acid

3 June 2018

1 reference

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The role of cysteine residues as redox-sensitive regulatory switches

3 June 2018

1 reference

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

3 June 2018

1 reference

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

3 June 2018

1 reference

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

3 June 2018

1 reference

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Molecular structure and physiological functions of GABA(B) receptors

3 June 2018

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Liver-directed adenoviral gene transfer in murine succinate semialdehyde dehydrogenase deficiency.

3 June 2018

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Glucose inhibition of glucagon secretion from rat alpha-cells is mediated by GABA released from neighboring beta-cells

3 June 2018

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A GABA(B) mystery: the search for pharmacologically distinct GABA(B) receptors

3 June 2018

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Regulated exocytosis of GABA-containing synaptic-like microvesicles in pancreatic beta-cells.

3 June 2018

1 reference

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From the street to the brain: neurobiology of the recreational drug gamma-hydroxybutyric acid

3 June 2018

1 reference

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

3 June 2018

1 reference

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

3 June 2018

1 reference

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Succinic semialdehyde dehydrogenase: purification and properties of the enzyme from monkey brain

3 June 2018

1 reference

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Hydroxynonenal, toxic carbonyls, and Alzheimer disease

3 June 2018

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Murine succinate semialdehyde dehydrogenase deficiency

3 June 2018

1 reference

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Magnetic resonance spectroscopy of neurotransmitters in human brain.

3 June 2018

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Carbonyl toxicology and Alzheimer's disease

3 June 2018

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Structure of human succinic semialdehyde dehydrogenase gene: identification of promoter region and alternatively processed isoforms

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Modulation of the GABA(A)-gated chloride channel by reactive oxygen species

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Neurotransmitters and neuromodulators during early human development.

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Visual field defects and other ophthalmological disturbances associated with vigabatrin

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

GHB: a new and novel drug of abuse

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Prenatal diagnosis of succinic semialdehyde dehydrogenase deficiency: increased accuracy employing DNA, enzyme, and metabolite analyses.

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Evidence for a G protein-coupled gamma-hydroxybutyric acid receptor

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Gamma-hydroxybutyric acid as a signaling molecule in brain

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Effect of long-term vigabatrin administration on the immature rat brain

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

The GABA paradox: multiple roles as metabolite, neurotransmitter, and neurodifferentiative agent

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Aldehyde-protein adducts in the liver as a result of ethanol-induced oxidative stress

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Activation of stress signaling pathways by the end product of lipid peroxidation. 4-hydroxy-2-nonenal is a potential inducer of intracellular peroxide production

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Enhanced GABA release in cell-damaging conditions in the adult and developing mouse hippocampus

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

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

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

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

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Purification and characterization of a coenzyme-A-dependent succinate-semialdehyde dehydrogenase from Clostridium kluyveri

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Regulation of gamma-aminobutyric acid synthesis in the brain

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Molecular organization of the Escherichia coli gab cluster: nucleotide sequence of the structural genes gabD and gabP and expression of the GABA permease gene

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Aldehyde dehydrogenases: widespread structural and functional diversity within a shared framework

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Gamma-hydroxybutyrate: an overview of the pros and cons for it being a neurotransmitter and/or a useful therapeutic agent

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Gamma-aminobutyric acid (GABA) metabolism in mammalian neural and nonneural tissues

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Defective succinic semialdehyde dehydrogenase activity in 4-hydroxybutyric aciduria.

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

4-Hydroxybutyric aciduria: a new inborn error of metabolism. III. Enzymology and inheritance.

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Brain succinate semialdehyde. II. Changes in the developing rat brain

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Characterization of succinate semialdehyde dehydrogenase from rat brain.

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

The distribution of GABA-transaminase-dehydrogenase activity in the myenteric plexus of rat small intestine: a histochemical analysis.

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Electron-cytochemical localization of succinic semialdehyde dehydrogenase activity in Purkinje neurons and hepatocytes of the rat.

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Succinic semialdehyde dehydrogenase activity in bovine adrenal medulla and blood platelets: a comparative study with the brain enzyme.

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Human brain "high Km" aldehyde dehydrogenase: purification, characterization, and identification as NAD+ -dependent succinic semialdehyde dehydrogenase.

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

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

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

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

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

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

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

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

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Vigabatrin therapy in patient with succinic semialdehyde dehydrogenase deficiency.

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

In vivo cloning and characterization of the gabCTDP gene cluster of Escherichia coli K-12.

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

The first adult case with 4-hydroxybutyric aciduria.

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

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

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

GABA and glycine in synaptic vesicles: storage and transport characteristics.

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Identification of human liver aldehyde dehydrogenases that catalyze the oxidation of aldophosphamide and retinaldehyde

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Succinic semialdehyde dehydrogenase from mammalian brain: subunit analysis using polyclonal antiserum

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Metabolism of gamma-hydroxy-[1-14C] butyrate by rat brain: relationship to the Krebs cycle and metabolic compartmentation of amino acids

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Purification and properties of two succinate semialdehyde dehydrogenases from human brain.

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Studies on the control of 4-aminobutyrate metabolism in ‘synaptosomal’ and free rat brain mitochondria

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Identification of endogenous gamma-hydroxybutyrate in human and bovine brain and its regional distribution in human, guinea pig and rhesus monkey brain

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Gamma hydroxybutyrate in the monkey: I. Electroencephalographic, behavioral, and pharmacokinetic studies

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Purification and properties of rat brain succinic semialdehyde dehydrogenase

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Synaptic and non-synaptic mitochondria from rat brain: isolation and characterization

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Cysteine string protein-alpha prevents activity-dependent degeneration in GABAergic synapses.

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Crystal structure of non-redox regulated SSADH from Escherichia coli

27 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

[Succinic semialdehyde dehydrogenase deficiency: decrease in 4-OH-butyric acid levels with low doses of vigabatrin].

27 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

GABA synthesis in Schwann cells is induced by the neuroactive steroid allopregnanolone

27 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Possible GABAergic modulation in the protective effect of allopregnanolone on sleep deprivation-induced anxiety-like behavior and oxidative damage in mice

27 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

The centenary of Garrod's Croonian lectures.

27 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Succinic semialdehyde dehydrogenase from the parasitic cattle tick Rhipicephalus microplus: gene identification, biochemical characterization and comparison with the mouse ortholog

27 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Excitatory GABA in rodent developing neocortex in vitro

27 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Developmental profile and mechanisms of GABA-induced calcium signaling in hippocampal astrocytes

27 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Functional characterization of a Drosophila melanogaster succinic semialdehyde dehydrogenase and a non-specific aldehyde dehydrogenase

27 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Progesterone attenuates neuroleptic-induced orofacial dyskinesia via the activity of its metabolite, allopregnanolone, a positive GABA(A) modulating neurosteroid

27 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Evidence for oxidative stress in tissues derived from succinate semialdehyde dehydrogenase-deficient mice

27 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Vigabatrin improves paroxysmal dystonia in succinic semialdehyde dehydrogenase deficiency.

27 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

A human derived SSADH coding variant is replacing the ancestral allele shared with primates

27 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

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

27 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Succinic semialdehyde dehydrogenase deficiency: clinical, biochemical and molecular characterization of a new patient with severe phenotype and a novel mutation

27 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

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

27 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Candidate gene analysis of the succinic semialdehyde dehydrogenase gene (ALDH5A1) in patients with idiopathic generalized epilepsy and photosensitivity

27 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

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

27 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Effect of genetically caused excess of brain gamma-hydroxybutyric acid and GABA on sleep.

27 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Gamma-hydroxybutyric aciduria: a biochemist's education from a heritable disorder of GABA metabolism

27 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

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

27 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

4-Hydroxy-trans-2-nonenoic acid is a gamma-hydroxybutyrate receptor ligand in the cerebral cortex and hippocampus

27 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

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

27 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

The molecular basis of succinic semialdehyde dehydrogenase deficiency in one family

27 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Monitoring gamma-hydroxybutyric acid levels in succinate-semialdehyde dehydrogenase deficiency.

27 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Bi-directional effects of GABA(B) receptor agonists on the mesolimbic dopamine system

27 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Specific gamma-hydroxybutyrate-binding sites but loss of pharmacological effects of gamma-hydroxybutyrate in GABA(B)(1)-deficient mice

27 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

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

27 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Succinic semialdehyde oxidation by a soluble dehydrogenase from brain.

27 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

GABA transporter-1 (GAT1)-deficient mice: differential tonic activation of GABAA versus GABAB receptors in the hippocampus.

27 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Clinical spectrum of succinic semialdehyde dehydrogenase deficiency

27 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Selective involvement of the globus pallidus and dentate nucleus in succinic semialdehyde dehydrogenase deficiency

27 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Visual field constriction in 91 Finnish children treated with vigabatrin

27 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Elevation of AKR7A2 (succinic semialdehyde reductase) in neurodegenerative disease

27 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Clinical, fluorine-18 labeled 2-fluoro-2-deoxyglucose positron emission tomography (FDG PET), MRI of the brain and biochemical observations in a patient with 4-hydroxybutyric aciduria; a progressive neurometabolic disease.

27 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

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

27 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Plant succinic semialdehyde dehydrogenase. Cloning, purification, localization in mitochondria, and regulation by adenine nucleotides.

27 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Synthesis of vesicular GABA from glutamine involves TCA cycle metabolism in neocortical neurons.

27 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Role of oxidative stress and the glutathione system in loss of dopamine neurons due to impairment of energy metabolism.

27 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Vigabatrin-associated retinal cone system dysfunction: electroretinogram and ophthalmologic findings.

27 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Enzymatic and immunological demonstration of normal and defective succinic semialdehyde dehydrogenase activity in fetal brain, liver and kidney.

27 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Vigabatrin therapy in six patients with succinic semialdehyde dehydrogenase deficiency.

27 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Regional and Subcellular Localization in Rat Brain of the Enzymes That Can Synthesize γ‐Hydroxybutyric Acid

27 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

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

27 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3125545

Purification and properties of two succinic semialdehyde dehydrogenases from Klebsiella pneumoniae

27 November 2018

1 reference

12 December 2020

Succinic semialdehyde dehydrogenase (SSADH) deficiency: Molecular analysis in a South American family

1 reference

12 December 2020

Activity of biotin-dependent and GABA metabolizing enzymes in chorionic villus samples: potential for 1st trimester prenatal diagnosis

1 reference

12 December 2020

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

1 reference

12 December 2020

Gamma-hydroxybutyric acid: an endogenous neuromodulator with abuse potential?

1 reference

12 December 2020

MRI findings in succinic semialdehyde dehydrogenase deficiency

1 reference

12 December 2020

Disturbed metabolism of guanidino compounds characterized by elevated excretion of beta-guanidinopropionic acid and gamma-guanidinobutyric acid--an effect of vigabatrin treatment?

1 reference

12 December 2020

A study of the effects of vigabatrin on the central nervous system and retina of Sprague Dawley and Lister-Hooded rats

1 reference

12 December 2020

Inborn errors of GABA metabolism

1 reference

12 December 2020

Use of ex vivo magnetic resonance imaging to detect onset of vigabatrin-induced intramyelinic edema in canine brain

1 reference

12 December 2020

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

1 reference

12 December 2020

Distribution of three enzymes of gamma-aminobutyric acid metabolism in monkey retina

1 reference

12 December 2020

Potato tuber succinate semialdehyde dehydrogenase: purification and characterization

1 reference

12 December 2020