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Rabbit Anti-NAV1.7/AP Conjugated antibody (bs-2427R-AP)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產(chǎn)品編號 bs-2427R-AP
英文名稱1 Rabbit Anti-NAV1.7/AP Conjugated antibody
中文名稱 堿性磷酸酶(AP)標(biāo)記的電壓開啟的鈉離子通道SCN9A抗體
別    名 SCN9A; Sodium channel protein type 9 subunit alpha; ETHA; hNE Na; hNENa; NE NA; NENA; NE-NA; Neuroendocrine sodium channel; Peripheral sodium channel 1; PN1; SCN9A; Sodium channel protein type 9 subunit alpha; Sodium channel protein type IX subunit alpha; Sodium channel voltage gated type IX alpha; Sodium channel voltage gated type IX alpha polypeptide; Sodium channel voltage gated type IX alpha subunit; Voltage gated sodium channel alpha subunit Nav1.7; Voltage gated sodium channel subunit alpha Nav1; FEB3B; SCN9A_HUMAN.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領(lǐng)域 通道蛋白  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Human,  (predicted: Mouse, Rat, Dog, Cow, Rabbit, )
產(chǎn)品應(yīng)用 WB=1:50-200 IHC-P=1:50-200 IHC-F=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 219kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human SCN9A
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(chǎn)品介紹 background:
This gene encodes a voltage-gated sodium channel which plays a significant role in nociception signaling. Mutations in this gene have been associated with primary erythermalgia, channelopathy-associated insensitivity to pain, and paroxysmal extreme pain disorder. [provided by RefSeq, Aug 2009]

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. It is a tetrodotoxin-sensitive Na(+) channel isoform. Plays a role in pain mechanisms, especially in the development of inflammatory pain.

Subunit:
The sodium channel consists of a large polypeptide and 2-3 smaller ones. This sequence represents a large polypeptide. Interacts with NEDD4 and NEDD4L.

Subcellular Location:
Membrane; Multi-pass membrane protein. Note=In neurite terminals.

Tissue Specificity:
Expressed strongly in dorsal root ganglion, with only minor levels elsewhere in the body, smooth muscle cells, MTC cell line and C-cell carcinoma. Isoform 1 is expressed preferentially in the central and peripheral nervous system. Isoform 2 is expressed preferentially in the dorsal root ganglion.

Post-translational modifications:
Ubiquitinated by NEDD4L; which may promote its endocytosis. Does not seem to be ubiquitinated by NEDD4.

DISEASE:
Defects in SCN9A are the cause of primary erythermalgia (PERYTHM) [MIM:133020]. It is an autosomal dominant disease characterized by recurrent episodes of severe pain associated with redness and warmth in the feet or hands.
Defects in SCN9A are the cause of congenital indifference to pain autosomal recessive (CIPAR) [MIM:243000]; also known as channelopathy-associated insensitivity to pain. A disorder characterized by congenital inability to perceive any form of pain, in any part of the body. All other sensory modalities are preserved and the peripheral and central nervous systems are apparently intact. Patients perceive the sensations of touch, warm and cold temperature, proprioception, tickle and pressure, but not painful stimuli. There is no evidence of a motor or sensory neuropathy, either axonal or demyelinating.
Defects in SCN9A are a cause of paroxysmal extreme pain disorder (PEPD) [MIM:167400]; previously known as familial rectal pain (FRP). PEPD is an autosomal dominant paroxysmal disorder of pain and autonomic dysfunction. The distinctive features are paroxysmal episodes of burning pain in the rectal, ocular, and mandibular areas accompanied by autonomic manifestations such as skin flushing.
Defects in SCN9A are a cause of generalized epilepsy with febrile seizures plus type 7 (GEFS+7) [MIM:613863]. GEFS+7 is 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. This disease combines 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 SCN9A are the cause of familial febrile convulsions type 3B (FEB3B) [MIM:613863]. FEB3B consists of seizures associated with febrile episodes in childhood without any evidence of intracranial infection or defined pathologic or traumatic cause. It is a common condition, affecting 2-5% of children aged 3 months to 5 years. The majority are simple febrile seizures (generally defined as generalized onset, single seizures with a duration of less than 30 minutes). Complex febrile seizures are characterized by focal onset, duration greater than 30 minutes, and/or more than one seizure in a 24 hour period. The likelihood of developing epilepsy following simple febrile seizures is low. Complex febrile seizures are associated with a moderately increased incidence of epilepsy.

Similarity:
Belongs to the sodium channel (TC 1.A.1.10) family.

Database links:

Entrez Gene: 6335 Human

Entrez Gene: 20274 Mouse

Entrez Gene: 78956 Rat

Omim: 603415 Human

SwissProt: Q15858 Human

SwissProt: Q62205 Mouse

SwissProt: O08562 Rat

Unigene: 439145 Human

Unigene: 440889 Mouse

Unigene: 88082 Rat



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

SCN9A是電壓門控鈉離子通道α-亞單位第Ⅸ型蛋白,是一種特殊的與疼痛有關(guān)的蛋白質(zhì),SCN9在機體中對鈉離子進入細(xì)胞以及神經(jīng)元之間的交流起導(dǎo)向作用。這種蛋白集中在痛覺神經(jīng)的末梢,當(dāng)人受到疼痛刺激時,這一蛋白質(zhì)會釋放鈉離子流,放大和刺激神經(jīng)細(xì)胞,將電子信號發(fā)送到大腦,從而使人們感覺到疼痛。
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