產(chǎn)品編號 yb-2390R
英文名稱 Anti-Aconitase 2抗體
中文名稱 鐵調(diào)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.
Anti-Aconitase 2抗體  
說 明 書 0.2ml  
研究領(lǐng)域 腫瘤  細(xì)胞生物  免疫學(xué)  染色質(zhì)和核信號  神經(jīng)生物學(xué)  線粒體  
抗體來源 Rabbit
克隆類型 Polyclonal
克 隆 號 85kDa
交叉反應(yīng) Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, 
產(chǎn)品應(yīng)用 WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 （石蠟切片需做抗原修復(fù)） 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 86kDa
細(xì)胞定位 細(xì)胞漿 線粒體
性    狀 Lyophilized or Liquid
濃    度 1mg/1ml
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.

PubMed PubMed
產(chǎn)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.


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