Succinic Semialdehyde Dehydrogenase: Biochemical–Molecular–Clinical Disease Mechanisms, Redox Regulation, and Functional Significance

Polysomnographic Abnormalities in Succinic Semialdehyde Dehydrogenase (SSADH) Deficiency

(2017) Phillip L. Pearl et al.   SLEEP

Neuropsychiatric Morbidity in Adolescent and Adult Succinic Semialdehyde Dehydrogenase Deficiency Patients

(2016) Ina Knerr et al.   CNS SPECTRUMS

The centenary of Garrod's Croonian lectures

(2013) D. J. Weatherall   CLINICAL MEDICINE

Crystal structure of non-redox regulated SSADH from Escherichia coli

(2010) Jae-Woo Ahn et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

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

(2010) Zita Dósa et al.   EPILEPSY RESEARCH

Molecular Identification of Carnosine Synthase as ATP-grasp Domain-containing Protein 1 (ATPGD1)

(2010) Jakub Drozak et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Cysteine String Protein-  Prevents Activity-Dependent Degeneration in GABAergic Synapses

(2010) P. Garcia-Junco-Clemente et al.   JOURNAL OF NEUROSCIENCE

Neuropathology in Succinic Semialdehyde Dehydrogenase Deficiency

(2010) Ina Knerr et al.   PEDIATRIC NEUROLOGY

Regulation of GABAA Receptor Subunit Expression by Pharmacological Agents

(2010) M. Uusi-Oukari et al.   PHARMACOLOGICAL REVIEWS

The X-Ray Crystal Structure of Escherichia coli Succinic Semialdehyde Dehydrogenase; Structural Insights into NADP+/Enzyme Interactions

(2010) Christopher G. Langendorf et al.   PLoS One

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

(2010) Evelyne Sernagor   Frontiers in Cellular Neuroscience

Déficit de succínico semialdehído deshidrogenasa. Disminución de los niveles de 4 OH butírico con dosis bajas de vigabatrina

(2009) G. Iglesias Escalera et al.   ANALES DE PEDIATRIA

Neuronal Glutamate and GABAAReceptor Function in Health and Disease

(2009) Peter R. Moult   BIOCHEMICAL SOCIETY TRANSACTIONS

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

(2009) Kirk Nylen et al.   BIOCHIMICA ET BIOPHYSICA ACTA-GENERAL SUBJECTS

Redox-switch modulation of human SSADH by dynamic catalytic loop

(2009) Yeon-Gil Kim et al.   EMBO JOURNAL

Evidence for genetic heterogeneity in D-2-hydroxyglutaric aciduria

(2009) Martijn Kranendijk et al.   HUMAN MUTATION

Succinic semialdehyde dehydrogenase deficiency: Lessons from mice and men

(2009) P. L. Pearl et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Visual evoked potentials in succinate semialdehyde dehydrogenase (SSADH) deficiency

(2009) G. Di Rosa et al.   JOURNAL OF INHERITED METABOLIC DISEASE

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

(2009) Valerio Magnaghi et al.   JOURNAL OF NEUROCHEMISTRY

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

(2009) A. Singh et al.   METHODS AND FINDINGS IN EXPERIMENTAL AND CLINICAL PHARMACOLOGY

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

(2009) P. L. Pearl et al.   NEUROLOGY

GABAA receptors and their associated proteins: Implications in the etiology and treatment of schizophrenia and related disorders

(2009) Erik I. Charych et al.   NEUROPHARMACOLOGY

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

(2009) Karsten K. Madsen et al.   PHARMACOLOGY & THERAPEUTICS

Narcolepsy and depression and the neurobiology of gammahydroxybutyrate

(2009) Mortimer Mamelak   PROGRESS IN NEUROBIOLOGY

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

(2009) A. K. Goodwin et al.   PSYCHOPHARMACOLOGY

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

(2009) Gordon Munro et al.   TRENDS IN PHARMACOLOGICAL SCIENCES

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

(2008) Erwin EW Jansen et al.   BMC DEVELOPMENTAL BIOLOGY

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

(2008) Angel Catalá   CHEMISTRY AND PHYSICS OF LIPIDS

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

(2008) Lee S. Stewart et al.   EPILEPSY & BEHAVIOR

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

(2008) Silke D. Meier et al.   GLIA

The Redox Environment in the Mitochondrial Intermembrane Space Is Maintained Separately from the Cytosol and Matrix

(2008) Jingjing Hu et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Garrod’s Croonian Lectures (1908) and the charter ‘Inborn Errors of Metabolism’: Albinism, alkaptonuria, cystinuria, and pentosuria at age 100 in 2008

(2008) Charles R. Scriver   JOURNAL OF INHERITED METABOLIC DISEASE

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

(2008) K. R. Drasbek et al.   JOURNAL OF INHERITED METABOLIC DISEASE

GABA, a natural immunomodulator of T lymphocytes

(2008) Helen Bjurstöm et al.   JOURNAL OF NEUROIMMUNOLOGY

Excitatory GABA in Rodent Developing Neocortex In Vitro

(2008) Sylvain Rheims et al.   JOURNAL OF NEUROPHYSIOLOGY

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

(2008) Boris Rothacker et al.   MOLECULAR AND BIOCHEMICAL PARASITOLOGY

GABAA receptors: Subtypes provide diversity of function and pharmacology

(2008) Richard W. Olsen et al.   NEUROPHARMACOLOGY

A ketogenic diet rescues the murine succinic semialdehyde dehydrogenase deficient phenotype

(2007) Kirk Nylen et al.   EXPERIMENTAL NEUROLOGY

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

(2007) B ROTHACKER et al.   INSECT BIOCHEMISTRY AND MOLECULAR BIOLOGY

Progesterone attenuates neuroleptic-induced orofacial dyskinesia via the activity of its metabolite, allopregnanolone, a positive GABAA modulating neurosteroid

(2007) Mahendra Bishnoi et al.   PROGRESS IN NEURO-PSYCHOPHARMACOLOGY & BIOLOGICAL PSYCHIATRY