Anti -GABA Receptor A, gamma 2 (GAG2)

For Research Use Only. Not for use in diagnostic procedures.

Chromosome: 5; NC_000005.9 (161494648..161582545). Location: 5q34

Polyclonal

IgG

Rabbit

Recognizes GABA receptor A, Gama 2. The 30 AA rat control peptide is 100% conserved in bovine, human and mouse GAG2 (2). It is ~80% conserved in chicken GAB2. Significant homology is also seen with rat GAG1 (64%), ratGAG3 (55%). It is not clear if anti-GAG21 actually reacts with other gamma subunits. No significant homology is observed with other GAA subunits (alpha, beta etc.). Antibody crossreactivity of anti-GAG21 in various species has also not been studied.

Serum
Serum

Supplied as a liquid, neat serum, 0.05% sodium azide, 40% glycerol.

May be stored at 4 degree C for short-term only. For long-term storage and to avoid repeated freezing and thawing, aliquot and add glycerol (40-50%). Freeze at -20 degree C. Aliquots are stable for at least 12 months at -20 degree C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.

Small volumes of anti-GABRG2 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

GABA (g-amino butyric acid) is the most abundant neurotransmitter in mammalian brain. GABA exerts its effects through ionotropic ligand-gated GABAA, GABAC and GABAB receptors (GABABRs). A family of GABA-A receptors subtypes exists, which are generated by alternative splicing of alpha 1-6, beta 1-4, gamma 1-4, delta, epsilon, pie, theta, and rho1-3 to form a heteromeric (pentameric) protein complexes. Various GABA-A subunits show distinct patterns of temporal and spatial expression that may imply its tissue specific physiological role (1). GABA A (GAA) receptor proteins (450-627 aa) are characterized by the presence of a cleavable signal peptide, a large extracellular N-terminus, 3 TM (transmembrane) domains, a large cytoplasmic domain followed by TM4 and C-terminal extracellular domain. The regions between TM3-4 and the large cytoplasmic loop are least conserved among various GAA subunits, which may confer subunit specific functionality. GAA genes are distributed as clusters throughout the human genome (chromosomes 4, 5, 15, and X; delta subunit on chromosome 1). GAA in the brain are the targets of many clinically important drugs.

Antibodies; Abs to Receptors

ELISA (EL/EIA), Western Blot (WB)

Suitable for use in ELISA, Western Blotting.
Dilution: Western Blotting: 1:1000-1:5000 for neat serum and 1-10ug/ml for affinity pure antibody using ECL.

ELISA
Control peptide can be used to coat ELISA plates at 1ug/ml and detected with antibodies (1:10-50K for neat serum and 0.5-1ug/ml for affinity pure).

54,162 Da[Similar Products]

gamma-aminobutyric acid (GABA) A receptor, gamma 2

gamma-aminobutyric acid receptor subunit gamma-2; OTTHUMP00000160873; OTTHUMP00000160874; OTTHUMP00000224398; OTTHUMP00000224400; GABA(A) receptor subunit gamma-2

Gamma-aminobutyric acid receptor subunit gamma-2

GBRG2_HUMAN

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]

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

Chromosomal Location of Human Ortholog: 5q34

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

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

Biological Process: synaptic transmission; transport; ***** 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

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