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Rabbit Anti-Aconitase 2/BF647 Conjugated antibody (bs-2390R-BF647)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-2390R-BF647
英文名稱 Rabbit Anti-Aconitase 2/BF647 Conjugated antibody
中文名稱 BF647標記的鐵調節(jié)蛋白2抗體
別    名 ACO 2; Aco-2; ACO2; ACON_HUMAN; aconitase 2; Aconitase 2 mitochondrial; Aconitase; aconitase-2; Aconitase2; Aconitate hydratase; Aconitate hydratase mitochondrial; ACONM; Citrate hydro lyase; Citrate hydro-lyase; ICRD; mitochondrial.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 腫瘤  細胞生物  免疫學  染色質和核信號  神經生物學  線粒體  
抗體來源 Rabbit
克隆類型 Polyclonal
克 隆 號 85kDa
交叉反應 (predicted: Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, )
產品應用 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 86kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Aconitase 2
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.
產品介紹 background:
The protein encoded by this gene belongs to the aconitase/IPM isomerase family. It is an enzyme that catalyzes the interconversion of citrate to isocitrate via cis-aconitate in the second step of the TCA cycle. This protein is encoded in the nucleus and functions in the mitochondrion. It was found to be one of the mitochondrial matrix proteins that are preferentially degraded by the serine protease 15(PRSS15), also known as Lon protease, after oxidative modification. [provided by RefSeq, Jul 2008]

Function:
Catalyzes the isomerization of citrate to isocitrate via cis-aconitate.

Subunit:
Monomer.

Subcellular Location:
Mitochondrion.

DISEASE:
Infantile cerebellar-retinal degeneration (ICRD) [MIM:614559]: A severe autosomal recessive neurodegenerative disorder characterized by onset between ages 2 and 6 months of truncal hypotonia, athetosis, seizures, and ophthalmologic abnormalities, particularly optic atrophy and retinal degeneration. Affected individuals show profound psychomotor retardation, with only some achieving rolling, sitting, or recognition of family. Brain MRI shows progressive cerebral and cerebellar degeneration. Note=The disease is caused by mutations affecting the gene represented in this entry.

Similarity:
Belongs to the aconitase/IPM isomerase family.

Database links:

Entrez Gene: 50 Human

Omim: 100850 Human

SwissProt: Q99798 Human

Unigene: 643610 Human



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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