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Rabbit Anti-SCN2A/BF647 Conjugated antibody (bs-1890R-BF647)
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说 明 书: 100ul  
100ul/2980.00元
大包装/询价
产品编号 bs-1890R-BF647
英文名称 Rabbit Anti-SCN2A/BF647 Conjugated antibody
中文名称 BF647标记的SCN2A抗体
别    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.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 细胞生物  免疫学  神经生物学  通道蛋白  细胞膜受体  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 (predicted: Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit, )
产品应用 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    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human SCN2A
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 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.
产品介绍 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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