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HRPBiotinGoldRBITCAPFITCCy3Cy5Cy5.5Cy7PEPE-Cy3PE-Cy5PE-Cy5.5PE-Cy7APCAlexa Fluor 350Alexa Fluor 488Alexa Fluor 555Alexa Fluor 594Alexa Fluor 647

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Anti-Nance-Horan Syndrome Protein/PE-Cy5.5

货号: bs-19007R-PE-Cy5.5 基本售价: 2980.0 元 规格: 100ul

产品信息

产品编号
bs-19007R-PE-Cy5.5
英文名称
Anti-Nance-Horan Syndrome Protein/PE-Cy5.5
中文名称
PE-Cy5.5标记的南斯-霍兰综合征蛋白抗体
别    名
Congenital cataracts and dental anomalies protein; CXN; Nance-Horan syndrome protein; nhs; NHS_HUMAN; RP3-389A20.6; SCML1.  
规格价格
100ul/2980元购买        大包装/询价
说 明 书
100ul  
研究领域
细胞生物  神经生物学  干细胞  
抗体来源
Rabbit
克隆类型
Polyclonal
交叉反应
Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit, Sheep, 
产品应用
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
179kDa
性    状
Lyophilized or Liquid
浓    度
1mg/ml
免 疫 原
KLH conjugated synthetic peptide derived from human Nance-Horan Syndrome Protein
亚    型
IgG
纯化方法
affinity purified by Protein A
储 存 液
0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件
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.
产品介绍
background:
This gene encodes a protein containing four conserved nuclear localization signals. The encoded protein functions in eye, tooth, craniofacial and brain development, and it can regulate actin remodeling and cell morphology. Mutations in this gene have been shown to cause Nance-Horan syndrome, and also X-linked cataract-40. Alternatively spliced transcript variants encoding different isoforms have been described for this gene. [provided by RefSeq, May 2014]

Function:
May function in cell morphology by maintaining the integrity of the circumferential actin ring and controlling lamellipod formation. Involved in the regulation eye, tooth, brain and craniofacial development.

Subunit:
Interacts with the tight junction protein TJP1/ZO-1. Associates with actin-rich structures. Interacts with BRK1 and with all three members of the WAVE protein family, WASF1, WASF2 and WASF3.

Subcellular Location:
Nucleus.

Tissue Specificity:
Detected at low levels in all tissues analyzed. Detected in fetal and adult brain, lens, retina, retinal pigment epithelium, placenta, lymphocytes and fibroblasts. Levels in retinal pigment epithelium, placenta, lymphocytes, and fibroblasts are very low. Expressed also in kidney, lung and thymus.

DISEASE:
Nance-Horan syndrome (NHS) [MIM:302350]: Rare X-linked disorder characterized by congenital cataracts, dental anomalies, dysmorphic features, and, in some cases, mental retardation. Distinctive dental anomalies are seen in affected males, including supernumerary incisors and crown shaped permanent teeth. Characteristic facial features are anteverted pinnae, long face, and prominent nasal bridge and nose. Carrier females display milder variable symptoms of disease with lens opacities often involving the posterior Y sutures, and on occasion dental anomalies and the characteristic facial features described.Note: The disease is caused by mutations affecting the gene represented in this entry.Cataract 40 (CTRCT40) [MIM:302200]: An opacification of the crystalline lens of the eye that frequently results in visual impairment or blindness. Opacities vary in morphology, are often confined to a portion of the lens, and may be static or progressive. CTRCT40 manifests as a congenital nuclear opacity with severe visual impairment in affected males. Heterozygous females have suture cataracts and only slight reduction in vision. In some cases, cataract is associated with microcornea without any other systemic anomaly or dysmorphism. Microcornea is defined by a corneal diameter inferior to 10 mm in both meridians in an otherwise normal eye.
Note: The disease is caused by mutations affecting the gene represented in this entry. Caused by copy number variations predicted to result in altered transcriptional regulation of the NHS gene: a 0.8 Mb segmental duplication-triplication encompassing the NHS, SCML1 and RAI2 genes, and an 4.8 kb intragenic deletion in NHS intron 1.

Similarity:
Belongs to the NHS family.

Database links:

Entrez Gene: 4810Human

Omim: 300457Human

SwissProt: Q6T4R5 Human

Unigene: 201623Human



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