亚洲制服欧美另类-午夜激情av电影-日本高清中文字幕一区二区三区-中国欧美日韩一区二区三区-欧洲亚洲日本韩国-成人欧美激情一区二区-亚洲偷偷自拍高清

掃碼關(guān)注公眾號           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
亚洲国产香蕉视频欧美,久久国产视频网站
Rabbit Anti-CD171/L1CAM/BF647 Conjugated antibody (bs-1996R-BF647)
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
產(chǎn)品編號 bs-1996R-BF647
英文名稱 Rabbit Anti-CD171/L1CAM/BF647 Conjugated antibody
中文名稱 BF647標(biāo)記的神經(jīng)細(xì)胞粘附分子配體1抗體
別    名 Antigen identified by monoclonal; R1; CAML1; CD171; CD171 antigen; HSAS; HSAS1; Hyd; L1; L1 cell adhesion molecule; L1-NCAM; L1cam; L1CAM_HUMAN; MASA; MIC5; N CAML1; N-CAM-L1; NCAM-L1; NCAM-L1; NCAML1; Nerve-growth factor-inducible large external glycoprotein; Neural cell adhesion molecule L1; NILE; OTTHUMP00000025992; S10; SPG1.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領(lǐng)域 發(fā)育生物學(xué)  神經(jīng)生物學(xué)  信號轉(zhuǎn)導(dǎo)  生長因子和激素  細(xì)胞骨架  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) (predicted: Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit, )
產(chǎn)品應(yīng)用 Flow-Cyt=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 138kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human CD171
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:
L1cam (L1 cell adhesion molecule isoform 1 precursor) is an axonal glycoprotein belonging to the immunoglobulin supergene family. The ectodomain, consisting of several immunoglobulin-like domains and fibronectin-like repeats (type III), is linked via a single transmembrane sequence to a conserved cytoplasmic domain. This cell adhesion molecule plays an important role in nervous system development, including neuronal migration and differentiation. Mutations in the gene cause three X-linked neurological syndromes known by the acronym CRASH (corpus callosum hypoplasia, retardation, aphasia, spastic paraplegia and hydrocephalus). Alternative splicing of a neuron-specific exon is thought to be functionally relevant. [provided by RefSeq].

Function:
Cell adhesion molecule with an important role in the development of the nervous system. Involved in neuron-neuron adhesion, neurite fasciculation, outgrowth of neurites, etc. Binds to axonin on neurons.

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

DISEASE:
Defects in L1CAM are the cause of hydrocephalus due to stenosis of the aqueduct of Sylvius (HSAS) [MIM:307000]. Hydrocephalus is a condition in which abnormal accumulation of cerebrospinal fluid in the brain causes increased intracranial pressure inside the skull. This is usually due to blockage of cerebrospinal fluid outflow in the brain ventricles or in the subarachnoid space at the base of the brain. In children is typically characterized by enlargement of the head, prominence of the forehead, brain atrophy, mental deterioration, and convulsions. In adults the syndrome includes incontinence, imbalance, and dementia. HSAS is characterized by mental retardation and enlarged brain ventricles.
Defects in L1CAM are the cause of mental retardation-aphasia-shuffling gait-adducted thumbs syndrome (MASA) [MIM:303350]; also known as corpus callosum hypoplasia, psychomotor retardation, adducted thumbs, spastic paraparesis, and hydrocephalus or CRASH syndrome. MASA is an X-linked recessive syndrome with a highly variable clinical spectrum. Main clinical features include spasticity and hyperreflexia of lower limbs, shuffling gait, mental retardation, aphasia and adducted thumbs. The features of spasticity have been referred to as complicated spastic paraplegia type 1 (SPG1). Some patients manifest corpus callosum hypoplasia and hydrocephalus. Inter- and intrafamilial variability is very wide, such that patients with hydrocephalus, MASA, SPG1, and agenesis of corpus callosum can be present within the same family.
Defects in L1CAM are the cause of spastic paraplegia X-linked type 1 (SPG1) [MIM:303350]. Spastic paraplegia is a degenerative spinal cord disorder characterized by a slow, gradual, progressive weakness and spasticity of the lower limbs.
Note=Defects in L1CAM may contribute to Hirschsprung disease by modifying the effects of Hirschsprung disease-associated genes to cause intestinal aganglionosis.
Defects in L1CAM are a cause of partial agenesis of the corpus callosum (ACCPX) [MIM:304100]. A syndrome characterized by partial corpus callosum agenesis, hypoplasia of inferior vermis and cerebellum, mental retardation, seizures and spasticity. Other features include microcephaly, unusual facies, and Hirschsprung disease in some patients.

Similarity:
Belongs to the immunoglobulin superfamily. L1/neurofascin/NgCAM family.
Contains 5 fibronectin type-III domains.
Contains 6 Ig-like C2-type (immunoglobulin-like) domains.

Database links:

Entrez Gene: 3897 Human

Entrez Gene: 16728 Mouse

Entrez Gene: 50687 Rat

Omim: 308840 Human

SwissProt: P32004 Human

SwissProt: P11627 Mouse

SwissProt: Q05695 Rat

Unigene: 522818 Human

Unigene: 260568 Mouse

Unigene: 10378 Rat



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

NCAM-L1又稱L1細(xì)胞粘附分子(L1CAM)是一種細(xì)胞表面糖蛋白,屬于免疫球蛋白超家族的神經(jīng)細(xì)胞粘附分子家族。L1CAM可能在神經(jīng)系統(tǒng)發(fā)育過程中發(fā)揮作用。
版權(quán)所有 2004-2026 a6308.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: 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: CQC24QY10047R0M/1100
京ICP備05066980號-1         京公網(wǎng)安備110107000727號
亚洲AV无码乱码在线观看侵犯 | 国产精品V日韩精品v | 日本xXXx色视频在线播放 | 18岁日韩内射颜射午夜久久成人 | 国产色综合色产在线视频 | 国产精品你当然比游戏重要 | 韩国三级理论在线电影 | XX性欧美肥妇精品久久久久久 | 少妇人妻偷人精品无码视频新浪 | 在线欧美精品一区二区三区 | 玩白嫩少妇小泬高潮18P | 一区二区三区在线观看视频 | 一二三四中文字幕 | 国产精品久久久久久久久久影院 | 国产97人人超碰cao蜜芽国产 | 国产无码视频在线观看 | 亚州免费一级毛片 | 欧美成a人片在线观看久 | 亚洲中文字幕无码中文字 | 欧美激情性A片视频播放 | 日韩一区精品视频一区二区 | 亚洲国产综合久久久精品 | 久久久精品中文字幕麻豆发布 | 女老板又大又紧水又多 | 精品国产青草久久久久福利 | 九九精品免费观看在线 | 内射人妻精品丨色AV无码 | 最火的app排行榜前十名 | 国产精品k频道在线看 | 亚洲精品拍拍央视网出文 | 成全动漫视频在线观看完整版 | 国产美女裸体无遮挡免费视频高潮 | 免费国产黄网站在线观看可以下载 | 亚洲欧美中文字幕5发布 | 最新黄色网址在线观看 | 好男人社区神马在线观看WWW | 青草草在线视频永久免费 | 国产东北三老头伦一肥婆 | 国产高潮婬乱叫床一区二区 | 日本午夜精品一区二区三区电影 | 欧美特黄特黄免费三级视频 |