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Rabbit Anti-SCN2A/BF555 Conjugated antibody (bs-1890R-BF555)
訂購熱線:400-901-9800
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訂購QQ:  400-901-9800
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-1890R-BF555
英文名稱 Rabbit Anti-SCN2A/BF555 Conjugated antibody
中文名稱 BF555標記的SCN2A抗體
別    名 sodium channel voltage-gated type II alpha; sodium channel, voltage-gated, type II, alpha isoform 2; HBSC II; NAC2; Scn2a; SCN2A_HUMAN; Sodium channel protein type II subunit alpha; Sodium channel protein type 2 subunit alpha; Sodium channel protein type II subunit alpha; Sodium channel protein, brain II subunit alph; Voltage-gated sodium channel subunit alpha Nav1.2; Voltage gated sodium channel subunit alpha Nav1.2; BFIC3; HBA; HBSCI; HBSCII; Na(v)1.2; NAC2; Nav1.2; SCN2A1; SCN2A2.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 細胞生物  免疫學  神經生物學  通道蛋白  細胞膜受體  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit, )
產品應用 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 228kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human SCN2A
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 °C 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°C. 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 °C.
產品介紹 background:
Voltage-gated sodium channels are transmembrane glycoprotein complexes composed of a large alpha subunit with 24 transmembrane domains and one or more regulatory beta subunits. They are responsible for the generation and propagation of action potentials in neurons and muscle. This gene encodes one member of the sodium channel alpha subunit gene family. It is heterogeneously expressed in the brain, and mutations in this gene have been linked to several seizure disorders. Several alternatively spliced transcript variants of this gene have been described, but the full-length nature of some of these variants has not been determined. [provided by RefSeq, Jul 2008]

Function:
Mediates the voltage-dependent sodium ion permeability of excitable membranes. Assuming opened or closed conformations in response to the voltage difference across the membrane, the protein forms a sodium-selective channel through which Na(+) ions may pass in accordance with their electrochemical gradient.

Subunit:
The sodium channel consists of a large polypeptide and 2-3 smaller ones. This sequence represents a large polypeptide. Interacts with NEDD4L (By similarity).

Subcellular Location:
Membrane; Multi-pass membrane protein.

Post-translational modifications:
May be ubiquitinated by NEDD4L; which would promote its endocytosis (By similarity).

DISEASE:
Defects in SCN2A are the cause of benign familial infantile convulsions type 3 (BFIC3) [MIM:607745]. BFIC3 is an autosomal dominant disorder in which afebrile seizures occur in clusters during the first year of life, without neurologic sequelae.
Defects in SCN2A are the cause of epileptic encephalopathy early infantile type 11 (EIEE11) [MIM:613721]. EIEE11 is an autosomal dominant seizure disorder characterized by infantile onset of refractory seizures with resultant delayed neurologic development and persistent neurologic abnormalities.

Similarity:
Belongs to the sodium channel (TC 1.A.1.10) family. Nav1.2/SCN2A subfamily.
Contains 1 IQ domain.

Database links:

Entrez Gene: 6326 Human

Entrez Gene: 110876 Mouse

Entrez Gene: 24766 Rat

Omim: 182390 Human

SwissProt: Q99250 Human

SwissProt: P04775 Rat

Unigene: 93485 Human

Unigene: 220329 Mouse

Unigene: 89192 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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