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产品信息
H00005498-M02
产品规格
种属反应
Human
宿主/亚型
Mouse
/ IgG2a, kappa
分类
Monoclonal
类型
Antibody
克隆号
2C10
抗原
PPOX (AAH02357, 378 a.a. approximately 477 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
偶联物
形式
Liquid
浓度
0.5-1 mg/mL
纯化类型
Protein A
保存液
PBS, pH 7.4
内含物
no preservative
保存条件
-20° C, Avoid Freeze/Thaw Cycles
运输条件
Wet ice
产品详细信息
Product may be used with Western Blot (Transfected lysate).
Immunogen sequence: EASGCVLSQE LFQQRAQEAA ATQLGLKEMP SHCLVHLHKN CIPQYTLGHW QKLESARQFL TAHRLPLTLA GASYEGVAVN DCIESGRQAA VSVLGTEPNS
靶标信息
Protoporphyrinogen oxidase, the penultimate enzyme in the heme biosynthetic pathway, catalyzes the 6-electron oxidation of protoporphyrinogen IX to form protoporphyrin IX. The PPOX protein localizes to the inner membrane of mitochondria from various tissues, including heart, brain, placenta, lung, liver, skeletal muscle, kidney and pancreas. Genetic deficiency of PPOX results in variegate porphyria, a low penetrance, autosomal dominant disorder characterized by cutaneous photosensitivity and/or various neurological manifestations. The rare homozygous variant of VP is characterized by severe PPOX deficiency, and results in the onset of photosensitization by porphyrins in early childhood, skeletal abnormalities of the hand, and, less constantly, short stature, mental retardation and convulsions.
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生物信息学
蛋白别名: PPO; Protoporphyrinogen oxidase
基因别名: PPO; PPOX; V290M; VP
UniProt ID: (Human) P50336
Entrez Gene ID: (Human) 5498
