Anti-EPB41抗体The 4.1 gene family encodes a group of multifunctional cytoskeletal proteins （4.1R, 4.1G, 4.1N and 4.1B）,

产品编号 yb-13080R
英文名称Anti-EPB41抗体
中文名称 红细胞膜条带4.1蛋白抗体
别    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.
Anti-EPB41抗体  
说 明 书 0.2ml  
研究领域 细胞生物  信号转导  细胞骨架  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Pig, Cow, Horse, Rabbit, Sheep, Recombinant Fragment
产品应用 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 （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 97kDa
细胞定位 细胞核 细胞浆 
性    状 Lyophilized or Liquid
浓    度 1mg/1ml
免 疫 原 KLH conjugated synthetic peptide derived from human EPB41/4.1R
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.

PubMed PubMed
产品介绍 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.