钠离子通道β1抗体

英文名称    SCN1B    

中文名称    钠离子通道β1抗体    

别    名    GEFSP1; SCN1B; SCN1B_HUMAN; sodium channel beta 1 subunit; Sodium channel subunit beta 1; Sodium channel subunit beta-1; Sodium channel voltage gated type I beta.    

钠离子通道β1抗体     

说 明 书    0.1ml  0.2ml      

研究领域    神经生物学  信号转导  通道蛋白  细胞膜受体      

抗体来源    Rabbit    

克隆类型    Polyclonal    

交叉反应    Human, Mouse, Rat,     

产品应用    WB=1:100-500 ELISA=1:500-1000 IP=1:20-100 IHC-P=1:100-500 IHC-F=1:100-500 Flow-Cyt=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.    

分 子 量    22kDa    

细胞定位    细胞膜     

性    状    Lyophilized or Liquid    

浓    度    1mg/1ml    

免 疫 原    KLH conjugated synthetic peptide derived from human SCN1B    

亚    型    IgG    

纯化方法    affinity purified by Protein A    

储 存 液    Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4    

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.    

钠离子通道β1抗体   产品介绍    background:
Crucial in the assembly, expression, and functional modulation of the heterotrimeric complex of the sodium channel. The subunit beta-1 can modulate multiple alpha subunit isoforms from brain, skeletal muscle, and heart. Its association with neurofascin may target the sodium channels to the nodes of Ranvier of developing axons and retain these channels at the nodes in mature myelinated axons.
Tissue specificity; Abundantly expressed in skeletal muscle, heart and brain.

Function:
Crucial in the assembly, expression, and functional modulation of the heterotrimeric complex of the sodium channel. The subunit beta-1 can modulate multiple alpha subunit isoforms from brain, skeletal muscle, and heart. Its association with neurofascin may target the sodium channels to the nodes of Ranvier of developing axons and retain these channels at the nodes in mature myelinated axons.

Subunit:
The voltage-sensitive sodium channel consists of an ion conducting pore forming alpha-subunit regulated by one or more beta-1, beta-2 and beta-3. Beta-1 and beta-3 are non-covalently associated with alpha, while beta-2 is covalently linked by disulfide bonds. Beta-1 or beta-3 subunits associate with neurofascin. Associates with SCN10A (By similarity).

Subcellular Location:
Membrane; Single-pass type I membrane protein.

Tissue Specificity:
The overall expression of isoforms 1 and 2 is very similar. Isoform 1 is abundantly expressed in skeletal muscle, heart and brain. Isoform 2 is highly expressed in brain and skeletal muscle and present at a very low level in heart, placenta, lung, liver, kidney and pancreas. In brain, isoform 2 is most abundant in the cerebellum, followed by the cerebral cortex and occipital lobe, while isoform 1 levels are higher in the cortex compared to the cerebellum. Isoform 2 is expressed in many regions of the brain, including cerebellar Purkinje cells, cortex pyramidal neurons and many of the neuronal fibers throughout the brain (at protein level). Also detected in dorsal root ganglion, in fibers of the spinal nerve and in cortical neurons and their processes (at protein level).

DISEASE:
Defects in SCN1B are the cause of generalized epilepsy with febrile seizures plus type 1 (GEFS+1) [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 SCN1B are the cause of Brugada syndrome type 5 (BRGDA5) [MIM:612838]. A tachyarrhythmia characterized by right bundle branch block and ST segment elevation on an electrocardiogram (ECG). It can cause the ventricles to beat so fast that the blood is prevented from circulating efficiently in the body. When this situation occurs (called ventricular fibrillation), the individual will faint and may die in a few minutes if the heart is not reset.

Similarity:
Belongs to the sodium channel auxiliary subunit SCN1B (TC 8.A.17) family.
Contains 1 Ig-like C2-type (immunoglobulin-like) domain.

Database links:
 

UniProtKB/Swiss-Prot: Q07699.1

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

钠离子通道β1抗体   

hz-9807R ECRG4/C2orf40  食道癌相关基因4蛋白抗体

hz-9808R C2ORF25  2号染色体开放阅读框25抗体

hz-9809R C2orf60  2号染色体开放阅读框抗体

hz-9810R C2orf68  2号染色体开放阅读框68抗体

hz-9811R C2ORF47  2号染色体开放阅读框47抗体

hz-9812R TCF9/GCF  转录因子9抗体

hz-9813R EGFR5/CLEC14A  表皮生长因子受体5抗体

hz-9814R C2orf44  2号染色体开放阅读框44抗体

hz-9815R C2orf42  2号染色体开放阅读框42抗体

hz-9816R C2orf43  2号染色体开放阅读框43抗体

hz-9817R C2orf55  2号染色体开放阅读框55抗体

hz-9818R C2orf57  2号染色体开放阅读框57抗体

hz-9819R C2orf69  2号染色体开放阅读框69抗体

hz-1649R phospho-APP/ABPP(Tyr757)  磷酸化APP(Tyr757)淀粉样肽前体蛋白抗体

hz-9820R C2orf89  2号染色体开放阅读框89抗体

hz-9821R C2orf29  2号染色体开放阅读框29抗体

hz-9822R C3orf24  3号染色体开放阅读框24抗体

hz-9823R AAT1/C3orf15  3号染色体开放阅读框15抗体

hz-9824R C3orf17  3号染色体开放阅读框17抗体

hz-3028R phospho-APP/ABPP(Thr668)  磷酸化APP淀粉样肽前体蛋白抗体

hz-9825R C3orf18  3号染色体开放阅读框18抗体

hz-9826R C3orf21/XXYLT1  3号染色体开放阅读框21抗体

hz-5167R phospho-APP/ABPP (Thr743)  磷酸化APP淀粉样肽前体蛋白抗体

hz-5168R phospho-APP/ABPP (Ser730)  磷酸化APP淀粉样肽前体蛋白抗体

hz-9827R C3orf23  3号染色体开放阅读框23抗体

hz-9828R C3orf33  3号染色体开放阅读框33抗体

hz-5186R phospho-APP/ABPP(Thr743)  磷酸化APP淀粉样肽前体蛋白抗体

hz-9829R C3orf37  3号染色体开放阅读框37抗体

hz-9830R C3orf39  3号染色体开放阅读框39抗体

hz-1632R APPL1  衔接因子蛋白含pH域磷酸酪氨酸结合域和亮氨酸拉链蛋白1抗体

hz-9831R C3orf49  3号染色体开放阅读框49抗体

hz-9832R C3orf54  3号染色体开放阅读框54抗体

hz-9833R C3orf59/MB21D2  3号染色体开放阅读框59抗体

hz-9836R HLC8/Lung cancer related protein 8  肺癌相关蛋白8抗体