• 靶点：
• HEM6

• 简介：
• >>Porphyrin metabolism;>>Metabolic pathways;>>Biosynthesis of cofactors

• 基因名称：
• CPOX CPO CPX

• 蛋白名称：
• HEM6

• Human Gene Id：
• 1371

• Human Swiss Prot No：
• P36551

• Mouse Gene Id：
• 12892

• Mouse Swiss Prot No：
• P36552

• Rat Gene Id：
• 304024

• Rat Swiss Prot No：
• Q3B7D0

• 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

• 组成：
• Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.

• 来源：
• Polyclonal, Rabbit,IgG

• 稀释：
• WB 1：500-2000

• 纯化工艺：
• The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

• 浓度：
• 1 mg/ml

• 储存：
• -15°C to -25°C/1 year(Do not lower than -25°C)

• 分子量：
• 50kD

• 背景：
• 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

• 细胞定位：
• Mitochondrion intermembrane space.

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