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SCN1B Polyclonal Antibody
SCN1B Polyclonal Antibody
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SCN1B Polyclonal Antibody
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经销商客户: ¥214.5
实验室客户: ¥292.5
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商品属性

Target:SCN1B

Fields:Adrenergic signaling in cardiomyocytes

Gene Name:SCN1B

Protein Name:Sodium channel subunit beta-1

Human Gene Id:6324

Human Swiss Prot No:Q07699

Mouse Swiss Prot No:P97952

Rat Swiss Prot No:Q00954

Immunogen:Synthesized peptide derived from part region of human protein

Specificity:SCN1B Polyclonal Antibody detects endogenous levels of protein.

Formulation:Liquid in PBS containing 50% glycerol, and 0.02% sodium azide.

Source:Polyclonal, Rabbit,IgG

Dilution:WB 1:500-2000 ELISA 1:5000-20000

Purification:The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Concentration:1 mg/ml

Storage Stability:-15°C to -25°C/1 year(Do not lower than -25°C)

Observed Band(KD):23kD

Background: Voltage-gated sodium channels are heteromeric proteins that function in the generation and propagation of action potentials in muscle and neuronal cells. They are composed of one alpha and two beta subunits, where the alpha subunit provides channel activity and the beta-1 subunit modulates the kinetics of channel inactivation. This gene encodes a sodium channel beta-1 subunit. Mutations in this gene result in generalized epilepsy with febrile seizures plus, Brugada syndrome 5, and defects in cardiac conduction. Multiple transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Oct 2009],

Function: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.,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

Subcellular Location:[Isoform 1]: Cell membrane ; Single-pass type I membrane protein . Perikaryon . Cell projection . Cell projection, axon . Detected at nodes of Ranvier on the sciatic nerve. .; [Isoform 2]: Perikaryon . Cell projection . Secreted . Detected on Purkinje cells and their cell projections and on neuronal cell projections. .

Expression:The overall expression of isoform 1 and isoform 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).

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