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Rabbit Anti-phospho-GABRG2 (Ser366)/BF555 Conjugated antibody (bs-12079R-BF555)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-12079R-BF555
英文名稱1 Rabbit Anti-phospho-GABRG2 (Ser366)/BF555 Conjugated antibody
中文名稱 BF555標記的磷酸化γ氨基丁酸γ2受體抗體
別    名 p-GABAA Rγ2 (Ser 365); CAE 2; CAE2; ECA 2; ECA2; GABA(A) receptor subunit gamma 2; GABA(A) receptor subunit gamma-2; GABRG 2; GABRG2 antibody Gamma aminobutyric acid (GABA) A receptor gamma 2; Gamma aminobutyric acid A receptor gamma 2; Gamma aminobutyric acid receptor gamma 2 subunit; Gamma-aminobutyric acid receptor subunit gamma-2; Gamma-aminobutyric-acid receptor gamma-2 subunit; GBRG2_HUMAN; GEFSP 3; GEFSP3.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
產品類型 磷酸化抗體 
研究領域 神經生物學  通道蛋白  細胞膜受體  G蛋白偶聯受體  G蛋白信號  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Mouse, Rat,  (predicted: Human, Chicken, Dog, Pig, Cow, Horse, Sheep, )
產品應用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 50kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthesised phosphopeptide derived from human GABRG2 around the phosphorylation site of Ser366
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol
保存條件 Store at -20 癈 for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20癈. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 癈.
產品介紹 background:
GAD-65 and GAD-67, glutamate decarboxylases, function to catalyze the production of GABA (gamma-aminobutyric acid). In the central nervous system, GABA functions as the main inhibitory transmitter by increasing a Cl- (chloride) conductance that inhibits neuronal firing. GABA has been shown to activate both ionotropic (GABAA) and metabotropic (GABAB) receptors, as well as a third class of receptors called GABAC. The ?subunit of GABAA receptors are important for benzodiazepine binding and modulation of GABA-mediated Cl- current. GABAA R? is a 467 amino acid mulit-pass membrane protein localized to the postsynaptic cell membrane. Present as a pentamer with other GABAA receptor chains (Alpha, Beta, Gamma, Delta and Epsilon), the GABAA ligand-gated Cl- channels selectively complex with D5DR to enable mutual inhibitory functional interactions between the two receptor systems. Defects in the gene encoding GABAA R Gamma 2 have been found to be the cause of childhood absence epilepsy type 2, familial febrile convulsions type 8, generalized epilepsy with febrile seizures plus type 3 and severe myoclonic epilepsy in infancy.

Function:
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.

Subunit:
Generally pentameric. There are five types of GABA(A) receptor chains: alpha, beta, gamma, delta, and rho. Interacts with GABARAP.

Subcellular Location:
Cell junction, synapse, postsynaptic cell membrane; Multi-pass membrane protein. Cell membrane; Multi-pass membrane protein.

Post-translational modifications:
Palmitoylated by ZDHHC3/GODZ; which may affect presynaptic clustering and/or cell surface stability.

DISEASE:
Defects in GABRG2 are the cause of childhood absence epilepsy type 2 (ECA2) [MIM:607681]. 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) [MIM:611277]. 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) [MIM:604233]. 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) [MIM:607208]; 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.

Similarity:
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.

Database links:

Entrez Gene: 282240 Cow

Entrez Gene: 2566 Human

Entrez Gene: 14406 Mouse

Entrez Gene: 29709 Rat

Omim: 137164 Human

SwissProt: P22300 Cow

SwissProt: P18507 Human

SwissProt: P22723 Mouse

SwissProt: P18508 Rat

Unigene: 7195 Human

Unigene: 5309 Mouse

Unigene: 159942 Rat



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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