中文名稱    紅細胞膜條帶4.1蛋白抗體    

英文名稱    EPB41

別    名    4.1R; 41_HUMAN; Band 4.1; E41P; EL 1; EL1; Elliptocytosis 1; Elliptocytosis 1 RH linked; EPB 4.1; EPB 41; EPB4.1; Epb41; Erythrocyte membrane protein band 4.1 (elliptocytosis 1 RH linked); Erythrocyte membrane protein band 4.1; Erythrocyte surface protein band 4.1; HE; P4.1; Protein 4.1.    

供 應 商    遠慕生物     

研究領域    細胞生物  信號轉導  細胞骨架      

抗體來源    Rabbit    

克隆類型    Polyclonal    

交叉反應    Human, Mouse, Rat, Pig, Cow, Horse, Rabbit, Sheep, Recombinant Fragment    

產品應用    WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 紅細胞膜條帶4.1蛋白抗體（石蠟切片需做抗原修復） 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.    

分 子 量    97kDa    

細胞定位    細胞核 細胞漿     

性    狀    Lyophilized or Liquid    

濃    度    1mg/1ml    

免 疫 原    KLH conjugated synthetic peptide derived from human EPB41/4.1R    

亞    型    IgG    

純化方法    affinity purified by Protein A    

儲 存 液    Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4    

保存條件    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.    

紅細胞膜條帶4.1蛋白抗體產品介紹    background:

The 4.1 gene family encodes a group of multifunctional cytoskeletal proteins (4.1R, 4.1G, 4.1N and 4.1B), which are predominantly expressed in the nervous system. 4.1G is a protein that stabilizes spectrin-actin interactions and is associated with hereditary elliptocytosis. Red blood cell 4.1, designated 4.1R, is a multifunctional protein that is essential for maintaining erythrocyte shape and membrane mechanical properties. Both 4.1R and 4.1G are distributed in a unique pattern in the cerebellum and are believed to modulate the membrane mechanical properties of neuronal cells by promoting fodrin/actin association. 4.1N and 4.1B, designated EPB41L1 and EPB41L3, respectively, are strongly expressed in the brain. Antibodies to 4.1N have been reported to detect mulitple forms, each enriched in postsynaptic density preparations relative to brain homogenate. Antibodies to 4.1B have been reported to detect two forms.

Function:
Protein 4.1 is a major structural element of the erythrocyte membrane skeleton. It plays a key role in regulating membrane physical properties of mechanical stability and deformability by stabilizing spectrin-actin interaction. Recruits DLG1 to membranes.

Subcellular Location:
Cytoplasm > cytoskeleton. Cytoplasm > cell cortex. Nucleus.

Post-translational modifications:
Phosphorylated at multiple sites by different protein kinases and each phosphorylation event selectively modulates the protein's functions.
Phosphorylation on Tyr-660 reduces the ability of 4.1 to promote the assembly of the spectrin/actin/4.1 ternary complex.
O-glycosylated; contains N-acetylglucosamine side chains in the C-terminal domain.

DISEASE:
Defects in EPB41 are the cause of elliptocytosis type 1 (EL1) [MIM:611804]. EL1 is a Rhesus-linked form of hereditary elliptocytosis, a genetically heterogeneous, autosomal dominant, hematologic disorder. It is characterized by variable hemolytic anemia and elliptical or oval red cell shape. Defects in EPB41 are a cause of hereditary pyropoikilocytosis (HPP) [MIM:266140]. HPP is an autosomal recessive hematologic disorder characterized by hemolytic anemia, microspherocytosis, poikilocytosis, and an unusual thermal sensitivity of red cells.