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PPOX Antibody (PA5-117044) in IHC (P)
Immunohistochemistry analysis of PPOX in paraffin-embedded human colon carcinoma. Samples were incubated with PPOX Polyclonal antibody (Product # PA5-117044) using a dilution of 1:100 (40x lens). Perform microwave antigen retrieval with 10 mM PBS buffer pH 7.2 before commencing with IHC staining protocol.
产品信息
PA5-117044
产品规格
种属反应
Human,
Mouse
宿主/亚型
Rabbit
/ IgG
分类
Polyclonal
类型
Antibody
抗原
Recombinant fusion protein containing a sequence corresponding to amino acids 1-280 of human PPOX (NP_0011162361)
偶联物
形式
Liquid
浓度
0.47 mg/mL
纯化类型
Affinity chromatography
保存液
PBS, pH 7.3, with 50% glycerol
内含物
0.02% sodium azide
保存条件
-20° C, Avoid Freeze/Thaw Cycles
运输条件
Wet ice
RRID
AB_2901674
产品详细信息
Positive Samples: HepG2, Mouse spleen, Mouse liver, Mouse kidney
Immunogen sequence: MGRTVVVLGG GISGLAASYH LSRAPCPPKV VLVESSERLG GWIRSVRGPN GAIFELGPRG IRPAGALGAR TLLLVSELGL DSEVLPVRGD HPAAQNRFLY VGGALHALPT GLRGLLRPSP PFSKPLFWAG LRELTKPRGK EPDETVHSFA QRRLGPEVAS LAMDSLCRGV FAGNSRELSI RSCFPSLFQA EQTHRSILLG LLLGAGRTPQ PDSALIRQAL AERWSQWSLR GGLEMLPQAL ETHLTSRGVS VLRGQPVCGL SLQAEGRWKV SLRDSSLEAD
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.
仅用于科研。不用于诊断过程。未经明确授权不得转售。
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生物信息学
蛋白别名: PPO; protoporhyrinogen oxidase; Protoporphyrinogen oxidase
基因别名: PPO; PPOX; V290M; VP
UniProt ID: (Human) P50336, (Mouse) P51175
Entrez Gene ID: (Human) 5498, (Mouse) 19044
