| 產(chǎn)品編號 | bs-22918R |
| 英文名稱 | CD171/L1CAM Rabbit pAb |
| 中文名稱 | 神經(jīng)細(xì)胞粘附分子配體1抗體 |
| 別 名 | L1CAM_HUMAN; Neural cell adhesion molecule L1; CAML1; MIC5; N-CAM-L1; NCAM-L1; CD171; L1 cell adhesion molecule; S10; HSAS; MASA; SPG1; HSAS1; N-CAM-L1; |
| 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 |
| 克 隆 號 | |
| 交叉反應(yīng) | Mouse,Rat (predicted: Human) |
| 產(chǎn)品應(yīng)用 | WB=1:500-2000
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理論分子量 | 138 kDa |
| 檢測分子量 | |
| 細(xì)胞定位 | 細(xì)胞膜 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human CD171/L1CAM |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 緩 沖 液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 保存條件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事項 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| 產(chǎn)品介紹 |
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. SWISS: P32004 Gene ID: 3897 Database links: Entrez Gene: 3897 Human Entrez Gene: 16728 Mouse Omim: 308840 Human SwissProt: P32004 Human SwissProt: P11627 Mouse Unigene: 522818 Human Unigene: 260568 Mouse Unigene: 10378 Rat 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。 |
| 產(chǎn)品圖片 |
25 ug total protein per lane of various lysates (see on figure) probed with CD171/L1CAM polyclonal antibody, unconjugated (bs-22918R) at 1:1000 dilution and 4°C overnight incubation. Followed by conjugated secondary antibody incubation at r.t. for 60 min.
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