FITC标记的丝聚合蛋白/中间丝蛋白抗体
产品名称： FITC标记的丝聚合蛋白/中间丝蛋白抗体
英文名称： Anti-FLG/FITC
产品编号： HZ-1462R-FITC
产品价格： null
产品产地： 中国/上海
品牌商标： HZbscience
更新时间： 2023-08-17T10:24:20
使用范围： IF=1:50-200
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Rabbit Anti-FLG/FITC Conjugated antibody
FITC标记的丝聚合蛋白/中间丝蛋白抗体
| 英文名称 | Anti-FLG/FITC |
| 中文名称 | FITC标记的丝聚合蛋白/中间丝蛋白抗体 |
| 别 名 | filaggrin; Epidermal filaggrin; Profilaggrin; Filaggrin precursor; FILA_HUMAN; FILA_MOUSE. |
| 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 细胞类型标志物 细胞骨架 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Mouse, Rat, |
| 产品应用 | IF=1:50-200 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 mouse FLG |
| 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. 细胞膜受体（Membrane Receptors) 丝聚合蛋白是存在于人表皮角质形成细胞内的一种易溶于水的蛋白，其生成的天然保湿因子(natural moisturizing factor，NMF)对保持皮肤的水合作用及屏障功能至关重要。丝聚合蛋白基因(filaggrin，FLG)位于人类染色体1q21.3区，是表皮分化复合物基因簇的成员之一，与细胞膜形成及表皮终末分化密切相关。FLG基因突变引起的皮肤屏障功能障碍在AD发病中扮演着重要角色，丝聚合蛋白的减少和缺失，可能是引起AD等干燥性皮肤病的主要原因 |
该基因编码的蛋白质是一种中间丝相关蛋白，在哺乳动物表皮中聚集角蛋白中间丝。它最初被合成为多蛋白前体，profil.rin(由多个丝蛋白单元组成，每个单元324a a)，定位于角质透明颗粒中，随后被蛋白水解加工成单个功能性丝蛋白分子。该基因的突变与寻常鱼鳞病有关。[ RefSeq，DEC 2009 ]提供。