Succinic semialdehyde dehydrogenase (SSADH) deficiency is a rare neurometabolic disorder characterized by defective degradation of gamma-aminobutyric acid (GABA), major inhibitory neurotransmitter the brain. Children with SSADH present motor mental delay, intractable seizures, infantile onset hypotonia, speech disturbances, extrapyramidal symptoms and ataxia. This wide spectrum results from inc...

The monohydrate form of the neurotransmitter γ-amino butyric acid (GABA) has been crystallised in the 0.4-0.8 GPa pressure range, recovered to ambient pressure and then used as a seed. Theoretical calculations indicate that this hydrate is only thermodynamically favoured over the two anhydrous forms at high pressures.

The role of gamma amino butyric acid (GABA) release and inhibitory neurotransmission in regulating most behaviors remains unclear. The vesicular GABA transporter (VGAT) is required for the storage of GABA in synaptic vesicles and provides a potentially useful probe for inhibitory circuits. However, specific pharmacologic agents for VGAT are not available, and VGAT knockout mice are embryonicall...

STUDY OBJECTIVES To explore absolute concentrations of brain metabolites including gamma amino-butyric acid (GABA) in the medial prefrontal cortex and basal ganglia of young adults with narcolepsy. DESIGN Proton magnetic resonance (MR) spectroscopy centered on the medial prefrontal cortex and the basal ganglia was acquired. The absolute concentrations of brain metabolites including GABA and g...

Gamma-amino butyric acid (GABA) has been characterized as inhibitory neurotransmitter through chloride mediated channels in the adult nervous system. However, using gramicidin perforated patch-clamp recordings from rod bipolar cells dissociated from retinas of adult mice, we find that GABA is capable of inducing cell depolarization. Currents mediated by GABA(A) and GABA(C) receptors were furthe...

Background: Gamma -aminobutyric acid (GABA), a non-protein amino acid acts as an inhibitory neurotransmitter in the central nervous system of mammalians. The glutamate decarboxylase (GAD) is responsible for the conversion of L-glutamate to GABA. The human brain has two isoforms of this enzyme, GAD65 and GAD67 that differ in molecular weight, amino acid sequence, antigenicity, cellular location ...

In the developing visual system of Xenopus laevis retinal ganglion cell (RGC) axons extend through the brain towards their major target in the midbrain, the optic tectum. Enroute, the axons are guided along their pathway by cues in the environment. In vitro, neurotransmitters have been shown to act chemotropically to influence the trajectory of extending axons and regulate the outgrowth of deve...

Succinic semialdehyde dehydrogenase enzyme (SSADH) deficiency is a rare autosomal disorder involving the catabolism of the neurotransmitter γ-amino butyric acid (GABA). In the absence of SSADH, GABA is converted to 4-hydroxybutyrate. 4-hydroxybutyrate, which is regarded as a biochemical hallmark, accumulating in serum, urine and cerebrospinal fluid. We reported three brothers whose parents were...

background: gamma -aminobutyric acid (gaba), a non-protein amino acid acts as an inhibitory neurotransmitter in the central nervous system of mammalians. the glutamate decarboxylase (gad) is responsible for the conversion of l-glutamate to gaba. the human brain has two isoforms of this enzyme, gad65 and gad67 that differ in molecular weight, amino acid sequence, antigenicity, cellular location ...

The presented work pioneers the embryonic Drosophila CNS for studies of the developmental regulation and function of gamma-amino butyric acid (GABA). We describe for the first time the developmental pattern of GABA in Drosophila and address underlying regulatory mechanisms. Surprisingly, and in contrast to vertebrates, detectable levels of GABA occur late during Drosophila neurogenesis, after e...