超碰97自拍_久久99久久98精品免观看软件_在线亚洲一区_九九视频这里只有精品_国产精品一区二区欧美视频_精品国产区一区

掃碼關(guān)注公眾號(hào)           掃碼咨詢技術(shù)支持           掃碼咨詢技術(shù)服務(wù)
  
客服熱線:400-901-9800  客服QQ:4009019800  技術(shù)答疑  技術(shù)支持  質(zhì)量反饋  關(guān)于我們  聯(lián)系我們
天天干天天av,好吊妞在线新免费视频,国产又色又爽又黄又免费软件
Rabbit Anti-SCN1A/Cy5.5 Conjugated antibody (bs-20129R-Cy5.5)
訂購熱線:400-901-9800
訂購郵箱:sales@bioss.com.cn
訂購QQ:  400-901-9800
技術(shù)支持:techsupport@bioss.com.cn
說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價(jià)
產(chǎn)品編號(hào) bs-20129R-Cy5.5
英文名稱 Rabbit Anti-SCN1A/Cy5.5 Conjugated antibody
中文名稱 Cy5.5標(biāo)記的SCN1A抗體
別    名 alpha; brain sodium channel type I; EIEE6; FEB3; FEB3A; FHM3; GEFS+2; GEFSP2; HBSC I; HBSCI; MIM 182390; NAC1; Nav 1.1; RBI; SCN1; Scn1a; SCN1A_HUMAN; SCN2A1; SMEI; sodium channel; Sodium channel protein brain I alpha subunit; Sodium channel protein brain I subunit alpha; Sodium channel protein type 1 subunit alpha; Sodium channel protein type I subunit alpha; Sodium channel voltage gated type 1 alpha subunit; Sodium channel voltage gated type I alpha polypeptide; type I; voltage gated; Voltage-gated sodium channel subunit alpha Nav1.1.  
規(guī)格價(jià)格 100ul/2980元 購買        大包裝/詢價(jià)
說 明 書 100ul  
研究領(lǐng)域 神經(jīng)生物學(xué)  通道蛋白  細(xì)胞膜受體  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) (predicted: Human, Mouse, Rat, Dog, Cow, Horse, Rabbit, Sheep, )
產(chǎn)品應(yīng)用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 229kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human SCN1A
亞    型 IgG
純化方法 affinity purified by Protein A
儲(chǔ) 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:
SCN1A is a voltage-gated ion channel essential for the generation and propagation of action potentials, mainly in nerve and muscle. Voltage-sensitive sodium channels are heteromeric complexes consisting of a large central pore-forming glycosylated alpha subunit, and two smaller auxiliary beta subunits. This gene encodes the large alpha subunit, and mutations in this gene have been associated with several epilepsy, convulsion and migraine disorders. Alternative splicing results in multiple transcript variants. The RefSeq Project has decided to create four representative RefSeq records. Three of the transcript variants are supported by experimental evidence and the fourth contains alternate 5' untranslated exons, the exact combination of which have not been experimentally confirmed for the full-length transcript

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.

Subcellular Location:
Membrane; Multi-pass membrane protein.

DISEASE:
Defects in SCN1A are the cause of generalized epilepsy with febrile seizures plus type 2 (GEFS+2) [MIM:604403]. 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 SCN1A are a cause of severe myoclonic epilepsy in infancy (SMEI) [MIM:607208]; also called Dravet syndrome. SMEI is a rare disorder characterized by generalized tonic, clonic, and tonic-clonic seizures that are initially induced by fever and begin during the first year of life. Later, patients also manifest other seizure types, including absence, myoclonic, and simple and complex partial seizures. Psychomotor development delay is observed around the second year of life. SMEI is considered to be the most severe phenotype within the spectrum of generalized epilepsies with febrile seizures-plus.
Defects in SCN1A are a cause of intractable childhood epilepsy with generalized tonic-clonic seizures (ICEGTC) [MIM:607208]. ICEGTC is a disorder characterized by generalized tonic-clonic seizures beginning usually in infancy and induced by fever. Seizures are associated with subsequent mental decline, as well as ataxia or hypotonia. ICEGTC is similar to SMEI, except for the absence of myoclonic seizures.
Defects in SCN1A are the cause of migraine familial hemiplegic type 3 (FHM3) [MIM:609634]. FHM3 is an autosomal dominant severe subtype of migraine with aura characterized by some degree of hemiparesis during the attacks. The episodes are associated with variable features of nausea, vomiting, photophobia, and phonophobia. Age at onset ranges from 6 to 15 years. FHM is occasionally associated with other neurologic symptoms such as cerebellar ataxia or epileptic seizures. A unique eye phenotype of elicited repetitive daily blindness has also been reported to be cosegregating with FHM in a single Swiss family.
Defects in SCN1A are the cause of familial febrile convulsions type 3A (FEB3A) [MIM:604403]; also known as familial febrile seizures 3. Febrile convulsions are 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. Nav1.1/SCN1A subfamily.
Contains 1 IQ domain.

Database links:

Entrez Gene: 6323 Human

Entrez Gene: 20265 Mouse

Entrez Gene: 81574 Rat

Omim: 182389 Human

SwissProt: P35498 Human

SwissProt: P04774 Rat

Unigene: 22654 Human

Unigene: 365737 Mouse

Unigene: 32079 Rat



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
版權(quán)所有 2004-2026 www.ggggww.cn 北京博奧森生物技術(shù)有限公司
通過國際質(zhì)量管理體系ISO 9001:2015 GB/T 19001-2016    證書編號(hào): 00124Q34771R2M/1100
通過國際醫(yī)療器械-質(zhì)量管理體系ISO 13485:2016 GB/T 42061-2022    證書編號(hào): CQC24QY10047R0M/1100
京ICP備05066980號(hào)-1         京公網(wǎng)安備110107000727號(hào)
主站蜘蛛池模板: 综合色综合 | 视频在线观看h | 久久久久亚洲AV成人网址 | 少妇厨房愉情理伦片BD在线观看 | 91原创国产 | 久久久精品精品 | 国产精品视频成人 | 春色校园亚洲愉拍自拍 | 正在播放白领少妇第一次 | 久久99精品国产自在现线小黄鸭 | 久久99精品国产自在现线小黄鸭 | 国产高清在线观看av | 一级做a爰性色毛片免费1 | 精品人妻无码一区二区三区换脸 | 在线观看国产精品一区 | 久久艹伊人 | 成全动漫在线观看免费观看 | 久久久精品理论A级A片 | 国产天堂第一区 | 老女人性生交大片免费 | 中文字幕在线视频免费视频 | 一级黄色免费观看 | 国产一区在线视频播放 | 欧美13一14娇小性 | 亚洲AV午夜成人片 | 中文字幕日韩欧美在线 | 国产AV无码专区亚洲AV潘金链 | 久久青青无码AV亚洲黑人 | 在线观看深夜视频 | 精品日本久久 | 波多野结衣在线观看一区二区 | 亚洲精品成人AA片在线播 | 女性女同性aⅴ免费观女性恋 | 国产精品探花一区在线观看 | 国产精品久久久久国产精品 | 久久69国产精品久久69软件 | 永久免费AV无码网站BT | 国产一级淫免费播放m | 天天摸夜夜摸爽爽狠狠婷婷97 | 亚洲精品无码aⅴ中文字幕蜜桃 | 国产精品综合AV一区二区国产馆 |