Rabbit GABA A Receptor gamma2 Polyclonal Antibody | anti-GABA antibody

GABA A Receptor gamma2 Rabbit pAb

Cat. Num. AAA8808399

• Gene Names: GABRG2; CAE2; ECA2; GEFSP3
• Reactivity: Human, Rat, Mouse
• Synonyms: GABA A Receptor gamma2; Polyclonal Antibody; GABA A Receptor gamma2 Rabbit pAb; CAE2;ECA2;GABRG2;GEFSP3; anti-GABA antibody

• Host: Rabbit
• Reactivity: Human, Rat, Mouse
• Clonality: Polyclonal
• Isotype: IgG
• Form/Format: PBS with 0.02% sodium azide and 50% glycerol pH7.4.
• Concentration: 1mg/ml (varies by lot)

• Species: Human
• Immunogen: Synthetic Peptide
• Preparation and Storage: Store at -20 degree C. Avoid repeated freeze-thaw cycles.

Western Blot (WB)

(Western blot analysis of 1) Mouse Brain Tissue, 2) Rat Brain Tissue with GABA A Receptor gamma2 Rabbit pAb EA289 diluted at 1:2,000.)

Immunohistochemistry (IHC)

(Immunohistochemical analysis of paraffin-embedded Rat BrainTissue using GABA A Receptor gamma2 (EA289)Rabbit pAb diluted at 1:200.)

Immunohistochemistry (IHC)

(Immunohistochemical analysis of paraffin-embedded Mouse BrainTissue using GABA A Receptor gamma2 (EA289)Rabbit pAb diluted at 1:200.)

Related Product Information for anti-GABA antibody

GABA, the major inhibitory neurotransmitter in the vertebrate brain, mediates neuronal inhibition by binding to the GABA/benzodiazepine receptor and opening an integral chloride channel.

NCBI and Uniprot Product Information

• NCBI GI #: 116242488
• NCBI GeneID: 2566
• UniProt Accession #: P18507; Q6GRL6; Q6PCC3; Q9UDB3; Q9UN15; F5HB82
• Molecular Weight: 467
• NCBI Official Full Name: Gamma-aminobutyric acid receptor subunit gamma-2
• NCBI Official Synonym Full Names: gamma-aminobutyric acid (GABA) A receptor, gamma 2
• NCBI Official Symbol: GABRG2
• NCBI Official Synonym Symbols: CAE2; ECA2; GEFSP3
• NCBI Protein Information: gamma-aminobutyric acid receptor subunit gamma-2; GABA(A) receptor, gamma 2; GABA(A) receptor subunit gamma-2
• UniProt Protein Name: Gamma-aminobutyric acid receptor subunit gamma-2
• UniProt Gene Name: GABRG2
• UniProt Entry Name: GBRG2_HUMAN

NCBI Description

This gene encodes a gamma-aminobutyric acid (GABA) receptor. GABA is the major inhibitory neurotransmitter in the mammlian brain, where it acts at GABA-A receptors, which are ligand-gated chloride channels. GABA-A receptors are pentameric, consisting of proteins from several subunit classes: alpha, beta, gamma, delta and rho. Mutations in this gene have been associated with epilepsy and febrile seizures. Multiple transcript variants encoding different isoforms have been identified for this gene. [provided by RefSeq, Jul 2008]

Uniprot Description

GABRG2: GABA, the major inhibitory neurotransmitter in the vertebrate brain, mediates neuronal inhibition by binding to the GABA/benzodiazepine receptor and opening an integral chloride channel. Defects in GABRG2 are the cause of childhood absence epilepsy type 2 (ECA2). ECA2 is a subtype of idiopathic generalized epilepsy (IGE) characterized by an onset at age 6-7 years, frequent absence seizures (several per day) and bilateral, synchronous, symmetric 3-Hz spike waves on EEG. During adolescence, tonic-clonic and myoclonic seizures develop. Some individuals manifest ECA2 occurring in combination with febrile convulsions. Defects in GABRG2 are the cause of familial febrile convulsions type 8 (FEB8). A febrile convulsion is defined as a seizure event in infancy or childhood, usually occurring between 6 months and 6 years of age, associated with fever but without any evidence of intracranial infection or defined pathologic or traumatic cause. Febrile convulsions affect 5-12% of infants and children up to 6 years of age. There is epidemiological evidence that febrile seizures are associated with subsequent afebrile and unprovoked seizures in 2% to 7% of patients. Defects in GABRG2 are the cause of generalized epilepsy with febrile seizures plus type 3 (GEFS+3). Generalized epilepsy with febrile seizures-plus refers to a rare autosomal dominant, familial condition with incomplete penetrance and large intrafamilial variability. Patients display febrile seizures persisting sometimes beyond the age of 6 years and/or a variety of afebrile seizure types. GEFS+ is a disease combining febrile seizures, generalized seizures often precipitated by fever at age 6 years or more, and partial seizures, with a variable degree of severity. Defects in GABRG2 are a cause of severe myoclonic epilepsy in infancy (SMEI); also called Dravet syndrome. SMEI is a rare disorder characterized by generalized tonic, clonic, and tonic-clonic seizures that are initially induced by fever and begin during the first year of life. Later, patients also manifest other seizure types, including absence, myoclonic, and simple and complex partial seizures. Psychomotor development delay is observed around the second year of life. SMEI is considered to be the most severe phenotype within the spectrum of generalized epilepsies with febrile seizures-plus. Belongs to the ligand-gated ion channel (TC 1.A.9) family. Gamma-aminobutyric acid receptor (TC 1.A.9.5) subfamily. GABRG2 sub-subfamily. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Membrane protein, multi-pass; Channel, chloride; Transporter, ion channel; Transporter; Membrane protein, integral; Channel, ligand-gated

Chromosomal Location of Human Ortholog: 5q34

Cellular Component: postsynaptic membrane; axon; integral to plasma membrane; cytoplasm; plasma membrane; cell junction

Molecular Function: chloride channel activity; protein binding; GABA-A receptor activity; benzodiazepine receptor activity; extracellular ligand-gated ion channel activity

Biological Process: synaptic transmission; transport; adult behavior; synaptic transmission, GABAergic; transmembrane transport; gamma-aminobutyric acid signaling pathway; post-embryonic development

Disease: Epilepsy, Childhood Absence, Susceptibility To, 2; Generalized Epilepsy With Febrile Seizures Plus, Type 3