Center for Bioinformatics
Oxidoreductases | Transferases | Hydrolases | Lyases | Isomerases | Ligases

Basic Information

Enzyme Number

Official Name

glutamate decarboxylase

Name from literature

glutamate decarboxylase

Pathway from literature

gama-amino butrylic acid (GABA) production

Pathway from KEGG

Amino Acid Metabolism; Glutamate metabolism; map00251

Human Diseases; Metabolic Disorders; Type I diabetes mellitus; map04940

Metabolism of Other Amino Acids; beta-Alanine metabolism; map00410

Amino Acid Metabolism; Alanine and aspartate metabolism; map00252

Carbohydrate Metabolism; Butanoate metabolism; map00650

Metabolism of Other Amino Acids; Taurine and hypotaurine metabolism; map00430


Human (9606)

Genome localization

2q31[2571 ], 10p11.23[2572 ],


A pyridoxal-phosphate protein. The brain enzyme also acts on L-cysteate, 3-sulfino-L-alanine and L-aspartate.

Rate-limiting Description

"We observed that a 48-hr treatment with the estrogen receptor antagonists ICI 182780 and tamoxifen decreased the level of glutamate decarboxylase (GAD)-65, a rate-limiting gamma-aminobutyric acid (GABA)-synthesizing enzyme, in a dissociated hippocampal neuronal culture." (17044036)

Regulatory Information

Upstream transcription factor


Regulatory type


transcriptional factor;REST(5978)

"This sequence census method was then used to map in vivo binding of the neuron-restrictive silencer factor (NRSF; also known as REST, for repressor element-1 silencing transcription factor) to 1946 locations in the human genome." (17540862)

key enzyme;

"Stiff-person syndrome is a rare autoimmune disorder associated with antibodies against glutamic acid decarboxylase (GAD), the key enzyme in gamma-aminobutyric acid synthesis." (15210535)


"Results describe the effect of phosphorylation on the two well-defined glutamic acid decarboxylase (GAD) isoforms, namely, GAD65 and GAD67, using highly purified preparations of recombinant human brain GAD65 and GAD67." (15147202)









Gene ontology

Gene ontology

GO:0019752 (P) carboxylic acid metabolic process [Q6ZSK1 ];
GO:0030054 (C) cell junction [Q05329 ];
GO:0004351 (F) glutamate decarboxylase activity [Q99259, Q6ZSK1, Q05329 ];
GO:0042136 (P) neurotransmitter biosynthetic process [Q99259, Q05329 ];
GO:0045202 (C) synapse [Q05329 ];
GO:0031410 (C) cytoplasmic vesicle [Q05329 ];
GO:0006540 (P) glutamate decarboxylation to succinate [Q99259, Q05329 ];
GO:0030170 (F) pyridoxal phosphate binding [Q99259, Q6ZSK1, Q05329 ];
GO:0018352 (P) protein-pyridoxal-5-phosphate linkage [Q99259 ];
GO:0005622 (C) intracellular [Q99259 ];
GO:0012506 (C) vesicle membrane [Q99259 ];
GO:0000139 (C) Golgi membrane [Q05329 ];
GO:0005515 (F) protein binding [Q99259, Q05329 ];

Tissue expression

Tissue From HPRD

Fetus [12011 ];
Frontal cortex [12011 ];
Islets of Langerhans [12011, 11817 ];
Pancreas [12011 ];
Testis [12011 ];
Adrenal cortex [12011 ];
Cerebellum [12011 ];
Brain [12011, 11817 ];

Tissue specificity

Isoform 3 is expressed in pancreatic islets, testis, adrenal cortex, and perhaps other endocrine tissues, but not in brain [Q99259 ];

Subcellular localization


synapse [Q05329 ];

golgi apparatus [Q05329 ];

membrane [Q05329 ];

cytoplasm [Q05329 ];

Disease relevance


Defects in GAD1 are the cause of autosomal recessive symmetric spastic cerebral palsy (SCP) [MIM:603513]. Cerebral palsy (CP) is an heterogeneous group of neurological disorders of movement and/or posture, with an estimated incidence of 1 in 250 to 1'000 live births, making CP one the commonest congenital disabilities. Non-progressive forms of symmetrical, spastic CP have been identified, which show a Mendelian autosomal recessive pattern of inheritance. Patients present developmental delay, mental retardation and sometimes epilepsy as part of the phenotype [Q99259 ];



Q05329; Q6ZSK1; Q99259

Entrez Gene

2571; 2572


12011; 11817

  Copyright 2009, Center for Bioinformatics 
  Last Modified: 2009-03-24  
  Design by Zhao Min