4-aminobutyrate transaminase: Difference between revisions

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{{Short description|Enzyme involved in the metabolism of gamma-aminobutyric acid}}
{{DISPLAYTITLE:4-Aminobutyrate Transaminase}}


'''4-aminobutyrate transaminase''' (also known as GABA transaminase) is an enzyme that plays a crucial role in the metabolism of the neurotransmitter [[gamma-aminobutyric acid]] (GABA). This enzyme is responsible for the conversion of GABA into succinic semialdehyde, which is a key step in the GABA shunt, a metabolic pathway that links the metabolism of GABA with the [[tricarboxylic acid cycle]].
== 4-Aminobutyrate Transaminase ==


==Structure==
[[File:4-aminobutyrate_transaminase_1ohv.jpg|thumb|right|Structure of 4-Aminobutyrate Transaminase]]
4-aminobutyrate transaminase is a [[pyridoxal phosphate]]-dependent enzyme, meaning it requires this cofactor to function. The enzyme is typically a homodimer, with each subunit contributing to the active site. The structure of 4-aminobutyrate transaminase has been elucidated through [[X-ray crystallography]], providing insights into its catalytic mechanism and substrate specificity.


==Function==
'''4-Aminobutyrate transaminase''' (also known as GABA transaminase) is an enzyme that plays a crucial role in the [[metabolism]] of the neurotransmitter [[gamma-aminobutyric acid]] (GABA). This enzyme is responsible for the conversion of GABA to [[succinate semialdehyde]], which is a key step in the [[GABA shunt]] pathway.
The primary function of 4-aminobutyrate transaminase is to catalyze the transamination reaction between GABA and [[alpha-ketoglutarate]], producing succinic semialdehyde and [[glutamate]]. This reaction is a part of the GABA shunt, which serves to recycle GABA and integrate it into central metabolic pathways. The GABA shunt is particularly important in the [[central nervous system]], where GABA acts as a major inhibitory neurotransmitter.


==Clinical significance==
== Function ==
Deficiencies or malfunctions in 4-aminobutyrate transaminase can lead to disorders such as [[GABA-transaminase deficiency]], which is characterized by elevated levels of GABA in the brain and associated neurological symptoms. Inhibitors of this enzyme, such as [[vigabatrin]], are used in the treatment of certain types of epilepsy, as they increase the levels of GABA in the brain by preventing its breakdown.
 
4-Aminobutyrate transaminase catalyzes the transfer of an amino group from GABA to [[alpha-ketoglutarate]], resulting in the production of succinate semialdehyde and [[glutamate]]. This reaction is an important part of the GABA shunt, which is a metabolic pathway that allows for the recycling of GABA and the production of energy in the form of [[ATP]].
 
== Structure ==
 
The enzyme is a [[pyridoxal phosphate]] (PLP)-dependent transaminase, meaning it requires PLP as a cofactor to function. The structure of 4-aminobutyrate transaminase has been elucidated through [[X-ray crystallography]], revealing a dimeric protein with each subunit containing a PLP-binding site.
 
== Clinical Significance ==
 
Dysfunction of 4-aminobutyrate transaminase can lead to disorders in GABA metabolism, which may result in neurological conditions such as [[epilepsy]] and [[schizophrenia]]. Inhibitors of this enzyme, such as [[vigabatrin]], are used in the treatment of epilepsy by increasing the levels of GABA in the brain.
 
== Related Enzymes ==
 
4-Aminobutyrate transaminase is part of a family of enzymes known as [[aminotransferases]], which are involved in the transfer of amino groups between molecules. Other related enzymes include [[alanine transaminase]] and [[aspartate transaminase]].
 
== Related Pages ==


==Related pages==
* [[Gamma-aminobutyric acid]]
* [[Gamma-aminobutyric acid]]
* [[GABA shunt]]
* [[Aminotransferase]]
* [[Pyridoxal phosphate]]
* [[Pyridoxal phosphate]]
* [[Tricarboxylic acid cycle]]
* [[Vigabatrin]]
==Gallery==
<gallery>
File:1ohv.jpg|Structure of 4-aminobutyrate transaminase
</gallery>


[[Category:Enzymes]]
[[Category:Enzymes]]
[[Category:Neurotransmitter metabolism]]
[[Category:Neurotransmitter metabolism]]
[[Category:Transaminases]]

Revision as of 11:48, 15 February 2025


4-Aminobutyrate Transaminase

File:4-aminobutyrate transaminase 1ohv.jpg
Structure of 4-Aminobutyrate Transaminase

4-Aminobutyrate transaminase (also known as GABA transaminase) is an enzyme that plays a crucial role in the metabolism of the neurotransmitter gamma-aminobutyric acid (GABA). This enzyme is responsible for the conversion of GABA to succinate semialdehyde, which is a key step in the GABA shunt pathway.

Function

4-Aminobutyrate transaminase catalyzes the transfer of an amino group from GABA to alpha-ketoglutarate, resulting in the production of succinate semialdehyde and glutamate. This reaction is an important part of the GABA shunt, which is a metabolic pathway that allows for the recycling of GABA and the production of energy in the form of ATP.

Structure

The enzyme is a pyridoxal phosphate (PLP)-dependent transaminase, meaning it requires PLP as a cofactor to function. The structure of 4-aminobutyrate transaminase has been elucidated through X-ray crystallography, revealing a dimeric protein with each subunit containing a PLP-binding site.

Clinical Significance

Dysfunction of 4-aminobutyrate transaminase can lead to disorders in GABA metabolism, which may result in neurological conditions such as epilepsy and schizophrenia. Inhibitors of this enzyme, such as vigabatrin, are used in the treatment of epilepsy by increasing the levels of GABA in the brain.

Related Enzymes

4-Aminobutyrate transaminase is part of a family of enzymes known as aminotransferases, which are involved in the transfer of amino groups between molecules. Other related enzymes include alanine transaminase and aspartate transaminase.

Related Pages