Anti-PPOX Antibody (CAB6397)
- SKU:
- CAB6397
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Metabolism
Frequently bought together:
Description
抗体名: | Anti-PPOX Antibody |
抗体コード: | CAB6397 |
抗体サイズ: | 20uL, 50uL, 100uL |
申し込み: | WB IHC IF |
反応性: | Human, Mouse |
宿主種: | Rabbit |
免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-280 of human PPOX (NP_001116236.1). |
申し込み: | WB IHC IF |
推奨希釈: | WB 1:500 - 1:2000 IHC 1:50 - 1:200 IF 1:50 - 1:100 |
反応性: | Human, Mouse |
ポジティブサンプル: | HepG2, Mouse spleen, Mouse liver, Mouse kidney |
免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-280 of human PPOX (NP_001116236.1). |
精製方法: | Affinity purification |
ストレージバッファ: | Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
アイソタイプ: | IgG |
順序: | MGRT VVVL GGGI SGLA ASYH LSRA PCPP KVVL VESS ERLG GWIR SVRG PNGA IFEL GPRG IRPA GALG ARTL LLVS ELGL DSEV LPVR GDHP AAQN RFLY VGGA LHAL PTGL RGLL RPSP PFSK PLFW AGLR ELTK PRGK EPDE TVHS FAQR RLGP EVAS LAMD SLCR GVFA GNSR ELSI RSCF PSLF QAEQ THRS ILLG LLLG AGRT PQPD SALI RQAL AERW SQWS LRGG LEML PQAL ETHL TSRG VSVL RGQP VCGL SLQA EGRW KVSL RDSS LEAD |
遺伝子ID: | 5498 |
Uniprot: | P50336 |
セルラーロケーション: | Intermembrane side, Mitochondrion inner membrane, Peripheral membrane protein |
計算された分子量: | 50kDa |
観察された分子量: | 51kDa |
同義語: | PPOX, PPO, V290M, VP |
バックグラウンド: | This gene encodes the penultimate enzyme of heme biosynthesis, which catalyzes the 6-electron oxidation of protoporphyrinogen IX to form protoporphyrin IX. Mutations in this gene cause variegate porphyria, an autosomal dominant disorder of heme metabolism resulting from a deficiency in protoporphyrinogen oxidase, an enzyme located on the inner mitochondrial membrane. Alternatively spliced transcript variants encoding the same protein have been identified. |
UniProt Protein Function: | PPOX: Catalyzes the 6-electron oxidation of protoporphyrinogen-IX to form protoporphyrin-IX. Defects in PPOX are the cause of variegate porphyria (VP). Porphyrias are inherited defects in the biosynthesis of heme, resulting in the accumulation and increased excretion of porphyrins or porphyrin precursors. They are classified as erythropoietic or hepatic, depending on whether the enzyme deficiency occurs in red blood cells or in the liver. PV is the most common form of porphyria in South Africa. It is characterized by skin hyperpigmentation and hypertrichosis, abdominal pain, tachycardia, hypertension and neuromuscular disturbances. High fecal levels of protoporphyrin and coproporphyrin, increased urine uroporphyrins and iron overload are typical markers of the disease. Belongs to the protoporphyrinogen oxidase family. |
UniProt Protein Details: | Protein type:Cofactor and Vitamin Metabolism - porphyrin and chlorophyll; Oxidoreductase; Mitochondrial; EC 1.3.3.4 Chromosomal Location of Human Ortholog: 1q22 Cellular Component: mitochondrial membrane; mitochondrial intermembrane space; integral to mitochondrial inner membrane; intrinsic to mitochondrial inner membrane Molecular Function:protoporphyrinogen oxidase activity; FAD binding Biological Process: response to drug; porphyrin metabolic process; protoporphyrinogen IX biosynthetic process; porphyrin biosynthetic process; heme biosynthetic process Disease: Porphyria Variegata |
NCBI Summary: | This gene encodes the penultimate enzyme of heme biosynthesis, which catalyzes the 6-electron oxidation of protoporphyrinogen IX to form protoporphyrin IX. Mutations in this gene cause variegate porphyria, an autosomal dominant disorder of heme metabolism resulting from a deficiency in protoporphyrinogen oxidase, an enzyme located on the inner mitochondrial membrane. Alternatively spliced transcript variants encoding the same protein have been identified. [provided by RefSeq, Jul 2008] |
UniProt Code: | P50336 |
NCBI GenInfo Identifier: | 1709742 |
NCBI Gene ID: | 5498 |
NCBI Accession: | P50336.1 |
UniProt Secondary Accession: | P50336,Q5VTW8, D3DVG0, |
UniProt Related Accession: | P50336 |
Molecular Weight: | 477 |
NCBI Full Name: | Protoporphyrinogen oxidase |
NCBI Synonym Full Names: | protoporphyrinogen oxidase |
NCBI Official Symbol: | PPOX |
NCBI Official Synonym Symbols: | VP; PPO; V290M |
NCBI Protein Information: | protoporphyrinogen oxidase |
UniProt Protein Name: | Protoporphyrinogen oxidase |
UniProt Gene Name: | PPOX |
UniProt Entry Name: | PPOX_HUMAN |
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