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Aconitase 2 Rabbit pAb (bs-2390R)  
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產(chǎn)品編號 bs-2390R
英文名稱 Aconitase 2 Rabbit pAb
中文名稱 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.  
研究領域 腫瘤  細胞生物  免疫學  染色質和核信號  神經(jīng)生物學  線粒體  
抗體來源 Rabbit
克隆類型 Polyclonal
克 隆 號
交叉反應 Mouse (predicted: Human,Rat,Rabbit,Pig,Cow,Dog,Horse)
產(chǎn)品應用 WB=1:500-2000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 86 kDa
檢測分子量
細胞定位 細胞漿 線粒體
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Aconitase 2: 651-750/780 
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 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 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.

SWISS:
Q99798

Gene ID:
50

Database links:

Entrez Gene: 50 Human

Omim: 100850 Human

SwissProt: Q99798 Human

Unigene: 643610 Human



產(chǎn)品圖片
Sample: Lane 1: Mouse Testis tissue lysates Lane 2: Mouse Kidney tissue lysates Lane 3: Mouse Large intestine tissue lysates Lane 4: Mouse Heart tissue lysates Primary: Anti-Aconitase 2 (bs-2390R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 86 kDa Observed band size: 82 kDa
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