铁调节蛋白2抗体
规格：1mg/1ml
英文名： Aconitase 2
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
分子量： 86kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human Aconitas
交叉反应：Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit,
细胞定位：细胞浆 线粒体
铁调节蛋白2抗体产品介绍：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 铁调节蛋白2抗体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. Gene ID: 50 Database links: Entrez Gene: 50 Human Omim: 100850 Human铁调节蛋白2抗体 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.
铁调节蛋白2抗体产品应用：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.
研究领域：肿瘤 细胞生物 **学 染色质和核信号 神经生物学 线粒体
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid