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EPB41 Rabbit pAb (bs-13080R)  
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產(chǎn)品編號(hào) bs-13080R
英文名稱 EPB41 Rabbit pAb
中文名稱 紅細(xì)胞膜條帶4.1蛋白抗體
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.  
研究領(lǐng)域 細(xì)胞生物  信號(hào)轉(zhuǎn)導(dǎo)  細(xì)胞骨架  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Rat (predicted: Human,Mouse,Rabbit,Pig,Sheep,Cow,Horse,Recombinant Fragment)
產(chǎn)品應(yīng)用 IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 97 kDa
檢測(cè)分子量
細(xì)胞定位 細(xì)胞核 細(xì)胞漿 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human EPB41/4.1R: 751-864/864 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項(xiàng) This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產(chǎn)品介紹 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.

Similarity:
Contains 1 FERM domain.

SWISS:
P11171

Gene ID:
2035

Database links:

Entrez Gene: 2035 Human

Entrez Gene: 269587 Mouse

Omim: 130500 Human

SwissProt: P11171 Human

SwissProt: P48193 Mouse

Unigene: 175437 Human

Unigene: 708933 Human

Unigene: 712722 Human

Unigene: 30038 Mouse



產(chǎn)品圖片
Tissue/cell: rat spleen tissue; 4% Paraformaldehyde-fixed and paraffin-embedded; Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat s
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