丝聚合蛋白/中间丝蛋白抗体

英文名称    FLG    

中文名称    丝聚合蛋白/中间丝蛋白抗体    

别    名    filaggrin; Epidermal filaggrin; Profilaggrin; Filaggrin precursor; FILA_HUMAN; FILA_MOUSE.    

说 明 书    0.1ml  0.2ml      

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    IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 丝聚合蛋白/中间丝蛋白抗体（石蜡切片需做抗原修复） 
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/1ml    

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 PBS, pH 7.4 with 10 mg/ml BSA and 0.1% Sodium azide    

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.    

丝聚合蛋白/中间丝蛋白抗体产品介绍    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.

Gene ID:
14246

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.     

丝聚合蛋白/中间丝蛋白抗体

A-43 RV IgM  风疹胞病毒 IgM(捕获法二步法)

A-16-1 TOXO IgG  弓形虫抗体 IgG(间接法二步法)

A-15-1 TOXO IgM  弓形虫抗体 IgM(间接法二步法)

A-33 TOXO IgM  弓形虫抗体 IgM(捕获法一步法)

A-44 TOXO IgM  弓形虫抗体 IgM(捕获法二步法)

A-40-1 HSV- I IgG  单纯疱疹Ⅰ型病毒 IgG(间接法二步法)

A-18-1 HSV- I IgM  单纯疱疹Ⅰ型病毒 IgM(间接法二步法)

A-36 HSV- I IgM  单纯疱疹Ⅰ型病毒 IgM（捕获法一步法）

A-45 HSV- I IgM  单纯疱疹Ⅰ型病毒 IgM（捕获法二步法）

A-41-1 HSV- II IgG  单纯疱疹Ⅱ型病毒 IgG(间接法二步法)

A-19-1 HSV- II IgM  单纯疱疹Ⅱ型病毒 IgM(间接法二步法)

A-37 HSV- II IgM  单纯疱疹Ⅱ型病毒 IgM （捕获法，一步法）

A-46 HSV- II IgM  单纯疱疹Ⅱ型病毒 IgM （捕获法，二步法）

E-73-48/96 Anti-Ins  胰岛素抗体（间接法二步法）

E-81-48/96 Anti-GAD  谷氨酸脱羧酶抗体（间接法二步法）

E-82-48/96 Ins  胰岛素（间接法二步法）

E-83-48/96 C-Peptide  C-肽（夹心法二步法）

E-91-48/96  破伤风抗体（双抗原夹心法）

A-31  包虫抗体IgG（间接法二步法）

hz-1012 DAO(Rat diamine oxidase,DAO) ELISA Kit  大鼠二胺氧化酶

hz-1012 Mouse anti-red cell antibody ELISA Kit   小鼠抗红细胞抗体

hz-1012 GCP-2/CXCL6(Human granulocyte chhzotactic protein-2,GCP-2) ELISA Kit  人粒细胞趋化蛋白-2

EA-1012 SAA (Rabbit Serum amyloid A) ELISA Kit  兔血清淀粉样蛋白A

EF-1012 C4 ELISA Kit   鲑鱼补体蛋白4

A-06-1 MV IgG  麻疹病毒 IgG（间接法二步法）

A-05-1 MV IgM  麻疹病毒 IgM（间接法二步法）

A-47-1 COX IgG  柯萨奇病毒 IgG 混合型（间接法二步法）

A-07-1 COX IgM  柯萨奇病毒 IgM 混合型(间接法二步法)

A-48-1 COX IgG I  柯萨奇病毒 IgG Ⅰ(间接法二步法)

A-08-1 COX IgM I  柯萨奇病毒 IgM Ⅰ(间接法二步法)

A-49-1 COX IgG II  柯萨奇病毒 IgG Ⅱ(间接法二步法)

A-50-1 COX IgG III  柯萨奇病毒 IgG Ⅲ(间接法二步法)

A-51-1 COX IgG IV  柯萨奇病毒 IgG Ⅳ(间接法二步法)