Anti-Aconitase 2抗体β1肾上腺素能受体（Beta-adrenergic Receptor-1 ）在接受交感神经节后纤维支配的各种器官中存在着与肾上腺素、*起反应的受体，称为肾上腺素能受体。

产品编号 yb-2390R
英文名称 Anti-Aconitase 2抗体
中文名称 铁调节蛋白2抗体
别    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抗体  
说 明 书 0.2ml  
研究领域 肿瘤  细胞生物  免疫学  染色质和核信号  神经生物学  线粒体  
抗体来源 Rabbit
克隆类型 Polyclonal
克 隆 号 85kDa
交叉反应 Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, 
产品应用 WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 86kDa
细胞定位 细胞浆 线粒体
性    状 Lyophilized or Liquid
浓    度 1mg/1ml
免 疫 原 KLH conjugated synthetic peptide derived from human Aconitase 2
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 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
产品介绍 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.