GABA alpha Ketoglutarate Aminotransferase, also known as GABA-T, is an enzyme that plays a crucial role in the metabolism of gamma-aminobutyric acid (GABA). It is a pyridoxal phosphate-dependent enzyme that catalyzes the transfer of an amino group from GABA to alpha-ketoglutarate, resulting in the formation of succinic semialdehyde and glutamate. This transfer reaction is biologically significant as it enables the conversion of GABA into a major excitatory neurotransmitter, glutamate.
GABA-T is primarily found in the brain, particularly in the mitochondria of neurons, where it participates in the GABA shunt pathway. The GABA shunt is an essential metabolic pathway that regulates the levels of GABA and glutamate in the brain, maintaining neurotransmitter homeostasis. By converting GABA into glutamate, GABA-T indirectly ensures the availability of glutamate as a precursor for the synthesis of neurotransmitters like glutamate, aspartate, and GABA itself.
Abnormalities or dysregulation of GABA-T activity can have significant implications for neurological health. GABA-T deficiency, for example, can lead to an accumulation of GABA and succinic semialdehyde, resulting in neurological disorders such as succinic semialdehyde dehydrogenase deficiency or GABA-transaminase deficiency. These conditions are characterized by symptoms like seizures, developmental delay, intellectual disability, and other neurological impairments.
In summary, GABA alpha Ketoglutarate Aminotransferase is an enzyme responsible for the metabolism of GABA, converting it into glutamate and succinic semialdehyde. It plays a crucial role in neurotransmitter homeostasis and its dysregulation can have
