GA(2) Ganglioside
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  GA(2) Ganglioside



GA(2) Ganglioside

   A specific monosialoganglioside that accumulates abnormally within the nervous system due to a deficiency of GM1-b-galactosidase, resulting in GM1 gangliosidosis.

RELATED TERMS
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Gangliosidosis
A group of often fatal inherited diseases marked by the accumulation of GANGLIOSIDES in LYSOSOMES secondary to enzymatic deficiency states. Gangliosidoses include TAY- SACHS DISEASE; GANGLIOSIDOSIS GM1; GANGLIOSIDOSES GM2; and SANDHOFF DISEASE; which share the infantile or childhood onset of CENTRAL NERVOUS SYSTEM deterioration. (Menkes, Textbook of Child Neurology, 5th ed, pp89-97)



SIMILAR TERMS
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PREVIOUS AND NEXT TERMS
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GABA B Receptors
Cell surface proteins which bind GAMMA-AMINOBUTYRIC ACID and influence cells via interactions with G-proteins. GABA-B receptors are pharmacologically characterized by their insensitivity to the blocker bicuculline and sensitivity to the agonist L-baclofen. They are found both presynaptically and postsynaptically, and act variously by inhibition of adenylate cyclase, activation of phospholipase A2, activation of potassium channels, and inactivation of voltage-activated calcium channels.

GABA Antagonists
Drugs that bind to but do not activate GAMMA-AMINOBUTYRIC ACID receptors, thereby blocking the actions of endogenous GAMMA-AMINOBUTYRIC ACID or GAMMA-AMINOBUTYRIC ACID agonists.

GABA Aminotransferase
An enzyme that converts brain gamma-aminobutyric acid (GAMMA-AMINOBUTYRIC ACID) into succinate semialdehyde, which can be converted to succinic acid and enter the citric acid cycle. It also acts on beta-alanine. EC 2.6.1.19.

GABA Agonists
Drugs that bind to and activate GAMMA-AMINOBUTYRIC ACID receptors (RECEPTORS, GABA).

GABA Agents
Substances used for their pharmacological actions on GABAergic systems. GABAergic agents include agonists, antagonists, degradation or uptake inhibitors, depleters, precursors, and modulators of receptor function.

GA(2) Ganglioside

GA-1 Germ Cell Antigen
A sex-specific cell surface antigen produced by the sex-determining gene of the Y chromosome in mammals. It causes syngeneic grafts from males to females to be rejected and interacts with somatic elements of the embryologic undifferentiated gonad to produce testicular organogenesis.

GABA, Chlorophenyl
A GAMMA-AMINOBUTYRIC ACID derivative that is a specific agonist at GABA-B receptors (RECEPTORS, GABA-B). It is used in the treatment of spasticity, especially that due to spinal cord damage. Its therapeutic effects result from actions at spinal and supraspinal sites, generally the reduction of excitatory transmission.

GABA Receptors
Cell-surface proteins that bind GAMMA-AMINOBUTYRIC ACID with high affinity and trigger changes that influence the behavior of cells. GABA-A receptors control chloride channels formed by the receptor complex itself. They are blocked by bicuculline and usually have modulatory sites sensitive to benzodiazepines and barbiturates. GABA-B receptors act through G-proteins on several effector systems, are insensitive to bicuculline, and have a high affinity for L-baclofen.

GABA Modulators
Substances that do not act as agonists or antagonists but do affect the GAMMA-AMINOBUTYRIC ACID receptor-ionophore complex. GABA-A receptors (RECEPTORS, GABA-A) appear to have at least three allosteric sites at which modulators act: a site at which benzodiazepines act by increasing the opening frequency of GAMMA-AMINOBUTYRIC ACID-activated chloride channels; a site at which barbiturates act to prolong the duration of channel opening; and a site at which some steroids may act. General anesthetics probably act at least partly by potentiating GABAergic responses, but they are not included here.

Gag Gene
DNA sequences that form the coding region for proteins associated with the viral core in retroviruses. gag is short for group-specific antigen.

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