扫码关注公众号           扫码咨询技术支持           扫码咨询技术服务
  
客服热线:400-901-9800  客服QQ:4009019800  技术答疑  技术支持  质量反馈  人才招聘  关于我们  联系我们
产品中心-北京博奥森生物技术有限公司
首页 > 产品中心 > 标记一抗 > 产品信息
Rabbit Anti-Filaggrin/Cy7 Conjugated antibody (bs-6327R-Cy7)
~~~促销,代码KXJ230206-6~~~
订购热线:400-901-9800
订购邮箱:sales@bioss.com.cn
订购QQ:  400-901-9800
技术支持:techsupport@bioss.com.cn
说 明 书: 100ul  
100ul/2980.00元
大包装/询价
产品编号 bs-6327R-Cy7
英文名称 Anti-Filaggrin/Cy7
中文名称 Cy7标记的丝聚蛋白/中间丝相关蛋白抗体
别    名 FLG; Epidermal filaggrin; Filaggrin precursor; Fillagrin; Profilaggrin; FILA_HUMAN.  
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  信号转导  细胞周期蛋白  细胞类型标志物  细胞骨架  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Rat,  (predicted: Mouse, Dog, Pig, Cow, Horse, Rabbit, )
产品应用
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 447kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Filaggrin
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:
The protein encoded by this gene is an intermediate filament-associated protein that aggregates keratin intermediate filaments in mammalian epidermis. It is initially synthesized as a polyprotein precursor, profilaggrin (consisting of multiple filaggrin units of 324 aa each), which is localized in keratohyalin granules, and is subsequently proteolytically processed into individual functional filaggrin molecules. Mutations in this gene are associated with ichthyosis vulgaris.[provided by RefSeq, Dec 2009].

Function:
Aggregates keratin intermediate filaments and promotes disulfide-bond formation among the intermediate filaments during terminal differentiation of mammalian epidermis.

Tissue Specificity:
Keratohyalin granules.

Post-translational modifications:
Filaggrin is initially synthesized as a large, insoluble, highly phosphorylated precursor containing many tandem copies of 324 AA, which are not separated by large linker sequences. During terminal differentiation it is dephosphorylated and proteolytically cleaved. The N-terminal of the mature protein is heterogeneous, and is blocked by the formation of pyroglutamate.
Undergoes deimination of some arginine residues (citrullination).

DISEASE:
Defects in FLG are the cause of ichthyosis vulgaris (VI) [MIM:146700]; also known as ichthyosis simplex. Ichthyosis vulgaris is the most common form of ichthyosis inherited as an autosomal dominant trait. It is characterized by palmar hyperlinearity, keratosis pilaris and a fine scale that is most prominent over the lower abdomen, arms, and legs. Ichthyosis vulgaris is characterized histologically by absent or reduced keratohyalin granules in the epidermis and mild hyperkeratosis. The disease can be associated with frequent asthma, eczema or hay fever.
Defects in FLG are a cause of susceptibility to dermatitis atopic type 2 (ATOD2) [MIM:605803]. Atopic dermatitis is a complex, inflammatory disease with multiple alleles at several loci thought to be involved in the pathogenesis. It commonly begins in infancy or early childhood and is characterized by a chronic relapsing form of skin inflammation, a disturbance of epidermal barrier function that culminates in dry skin, and IgE-mediated sensitization to food and environmental allergens. It is manifested by lichenification, excoriation, and crusting, mainly on the flexural surfaces of the elbow and knee.

Similarity:
Belongs to the S100-fused protein family.
Contains 2 EF-hand domains.
Contains 23 filaggrin repeats.

Database links:

Entrez Gene: 2312 Human

Entrez Gene: 14246 Mouse

Omim: 135940 Human

SwissProt: P20930 Human

SwissProt: P11088 Mouse

Unigene: 654510 Human

Unigene: 162684 Mouse

Unigene: 439710 Mouse



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
版权所有 2004-2023 www.bioss.com.cn 北京博奥森生物技术有限公司
通过国际质量管理体系ISO 9001:2015 GB/T 19001-2016    认证编号: 00122Q31509R1M/1100
京ICP备05066980号-1         京公网安备110107000727号