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Rabbit Anti-ETHE1/BF488 Conjugated antibody (bs-7610R-BF488)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產(chǎn)品編號 bs-7610R-BF488
英文名稱 Rabbit Anti-ETHE1/BF488 Conjugated antibody
中文名稱 BF488標記的乙基丙二酸腦病蛋白抗體
別    名 mitochondrial; Ethe1; ETHE1 protein, mitochondrial precursor; ETHE1_HUMAN; ethylmalonic encephalopathy 1; Ethylmalonic encephalopathy protein 1; hepatoma subtracted clone one; Hepatoma subtracted clone one protein; HSCO; Protein ETHE1; YF13H12.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 細胞生物  信號轉(zhuǎn)導  細胞凋亡  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Dog, Pig, Cow, Rabbit, Sheep, )
產(chǎn)品應用 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 26kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human ETHE1
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol
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.
產(chǎn)品介紹 background:
Probably plays an important role in metabolic homeostasis in mitochondria. May function as a nuclear-cytoplasmic shuttling protein that binds transcription factor RELA/NFKB3 in the nucleus and exports it to the cytoplasm. Suppresses p53-induced apoptosis by preventing nuclear localization of RELA.
Involvement in disease:Defects in ETHE1 are a cause of ethylmalonic encephalopathy (EE) . EE is an autosomal recessive disorder characterized by neurodevelopmental delay and regression, recurrent petechiae, acrocyanosis, diarrhea, leading to death in the first decade of life. It is also associated with persistent lactic acidemia and ethylmalonic and methylsuccinic aciduria.

Function:
Probably plays an important role in metabolic homeostasis in mitochondria. May function as a nuclear-cytoplasmic shuttling protein that binds transcription factor RELA/NFKB3 in the nucleus and exports it to the cytoplasm. Suppresses p53-induced apoptosis by preventing nuclear localization of RELA.

Subunit:
Interacts with RELA.

Subcellular Location:
Cytoplasm. Nucleus. Mitochondrion matrix. Note=According to PubMed:12398897, it is cytoplasmic and nuclear. According to PubMed:14732903, it is found in the mitochondrial matrix.

Tissue Specificity:
Ubiquitously expressed.

DISEASE:
Defects in ETHE1 are a cause of ethylmalonic encephalopathy (EE) [MIM:602473]. EE is an autosomal recessive disorder characterized by neurodevelopmental delay and regression, recurrent petechiae, acrocyanosis, diarrhea, leading to death in the first decade of life. It is also associated with persistent lactic acidemia and ethylmalonic and methylsuccinic aciduria.

Similarity:
Belongs to the metallo-beta-lactamase superfamily. Glyoxalase II family.

Database links:

Entrez Gene: 23474 Human

Entrez Gene: 66071 Mouse

Entrez Gene: 292710 Rat

Omim: 608451 Human

SwissProt: O95571 Human

SwissProt: Q9DCM0 Mouse

Unigene: 7486 Human

Unigene: 29553 Mouse

Unigene: 14691 Rat



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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