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HEM6 mouse mAb

BYmab-11189

  • 20UL ¥460 50UL ¥945 100UL ¥1350
  • 货期: 3个工作日

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宿主
反应性
应用
WB
分子量(DA)
免疫原
Synthesized peptide derived from human HEM6 AA range: 265-315
特异性
This antibody detects endogenous levels of HEM6 at Human/Mouse/Rat
来源
Monoclonal, Mouse,IgG
组成(Formulation)
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
稀释比例
WB 1:500-2000
纯化工艺(Immunogen)
The antibody was affinity-purified from mouse serum by affinity-chromatography using specific immunogen.
浓度
1 mg/ml
背景(Background)
The protein encoded by this gene is the sixth enzyme of the heme biosynthetic pathway. The encoded enzyme is soluble and found in the intermembrane space of mitochondria. This enzyme catalyzes the stepwise oxidative decarboxylation of coproporphyrinogen III to protoporphyrinogen IX, a precursor of heme. Defects in this gene are a cause of hereditary coproporphyria (HCP).[provided by RefSeq, Oct 2009],
功能
catalytic activity:Coproporphyrinogen-III + O(2) + 2 H(+) = protoporphyrinogen-IX + 2 CO(2) + 2 H(2)O.,disease:Defects in CPOX are the cause of hereditary coproporphyria (HCP) [MIM:121300]. HCP is an acute hepatic porphyria and an autosomal dominant disease characterized by neuropsychiatric disturbances and skin photosensitivity. Biochemically, there is an overexcretion of coproporphyrin III in the urine and in the feces. HCP is clinically characterized by attacks of abdominal pain, neurological disturbances, and psychiatric symptoms. The symptoms are generally manifested with rapid onset, and can be precipitated by drugs, alcohol, caloric deprivation, infection, endocrine factors or stress. A severe variant form is harderoporphyria, which is characterized by earlier onset attacks, massive excretion of harderoporphyrin in the feces, and a marked decrease of coproporphyrinogen IX oxidase
基因名称(Gene Name)
CPOX CPO CPX
蛋白名称
HEM6
简称
HEM6
其他名称
Fields
>>Porphyrin metabolism;>>Metabolic pathways;>>Biosynthesis of cofactors
人基因ID
人蛋白质序列数据库
小鼠基因ID
小鼠蛋白质序列数据库
大鼠基因ID
大鼠蛋白质序列数据库
细胞定位
Mitochondrion intermembrane space.
组织表达
储存(Storage)
-20°C/1 year

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HEM6 mouse mAb

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