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

掃碼關注公眾號           掃碼咨詢技術支持           掃碼咨詢技術服務
  
客服熱線:400-901-9800  客服QQ:4009019800  技術答疑  技術支持  質量反饋  關于我們  聯系我們
无遮免费网站在线入口,激情aa视频试看免费
首頁 > 產品中心 > 標記一抗 > 產品信息
Rabbit Anti-CD171/L1CAM/Cy5 Conjugated antibody (bs-1996R-Cy5)
訂購熱線:400-901-9800
訂購郵箱:sales@bioss.com.cn
訂購QQ:  400-901-9800
技術支持:techsupport@bioss.com.cn
說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-1996R-Cy5
英文名稱 Rabbit Anti-CD171/L1CAM/Cy5 Conjugated antibody
中文名稱 Cy5標記的神經細胞粘附分子配體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  
研究領域 發(fā)育生物學  神經生物學  信號轉導  生長因子和激素  細胞骨架  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit, )
產品應用 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細胞粘附分子(L1CAM)是一種細胞表面糖蛋白,屬于免疫球蛋白超家族的神經細胞粘附分子家族。L1CAM可能在神經系統發(fā)育過程中發(fā)揮作用。
版權所有 2004-2026 www.a6308.cn 北京博奧森生物技術有限公司
通過國際質量管理體系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         京公網安備110107000727號
亚洲免费视频在线 | 91嫖妓站街按摩店老熟女 | 最近中文字幕高清字幕MV | 国产成人午夜精品5599 | 午夜福利久久久噜久噜久久综合 | 无码成A毛片免费 | 麻豆一区二区免费播放网站 | 2018国产大陆天天弄 | 久久久久国产美女免费网站 | 国产男女性潮高清免费网站 | 亚洲精品欧美精品日韩精品 | 日日噜噜夜夜狠狠久久丁香五月 | 无码视频在线肉在线播放美熟女 | 精品国产一区二区三区无码黄 | 亚洲欧美精品一中文字幕 | 麻豆一二三区AV传媒 | 国产免费看JIZZ视频 | 91无码人妻精品一区 | 91精品夜夜夜一区二区 | 国产精品九九免费视频 | 国产国拍亚洲精品永久软件 | 亚洲中文乱码AV网站 | 少妇被躁爽到高潮无码麻豆AV | 国产精品爱啪在线播放 | 好爽毛片一区二区三区色欲 | 国产亚洲tv在线观看 | 打扑克污黄对网站在线看 | 亚洲欧洲自拍拍偷午夜色无码 | av无码精品久久久久精品免费 | 国产婷婷色一区二区三区在线 | 青草国产精品久久久久久 | 精品人妻无码一区二区三区在线 | a色毛片免费视频 | 国产精品v欧美精品v日韩精品 | 向日葵视频APP秋葵榴莲网站 | 国产精品久久久久久 | 国产一级欧美精品大片免费看 | 亚洲AV无码专区在线观看在线 | 情趣白丝女仆下部被揉小说合集 | 日韩一卡2卡3卡4卡新区亚洲 | 黄录像欧美片在线观看 |