Metabolism Antibodies 2
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
Description
| Antibody Name: | Anti-PPOX Antibody |
| Antibody SKU: | CAB6397 |
| Antibody Size: | 20uL, 50uL, 100uL |
| Application: | WB IHC IF |
| Reactivity: | Human, Mouse |
| Host Species: | Rabbit |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-280 of human PPOX (NP_001116236.1). |
| Application: | WB IHC IF |
| Recommended Dilution: | WB 1:500 - 1:2000 IHC 1:50 - 1:200 IF 1:50 - 1:100 |
| Reactivity: | Human, Mouse |
| Positive Samples: | HepG2, Mouse spleen, Mouse liver, Mouse kidney |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-280 of human PPOX (NP_001116236.1). |
| Purification Method: | Affinity purification |
| Storage Buffer: | Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
| Isotype: | IgG |
| Sequence: | 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 |
| Gene ID: | 5498 |
| Uniprot: | P50336 |
| Cellular Location: | Intermembrane side, Mitochondrion inner membrane, Peripheral membrane protein |
| Calculated MW: | 50kDa |
| Observed MW: | 51kDa |
| Synonyms: | PPOX, PPO, V290M, VP |
| Background: | 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 |
 | Western blot analysis of extracts of various cell lines, using PPOX Antibody (CAB6397) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Enhanced Kit (RM00021). Exposure time: 90s. |
 | Immunohistochemistry of paraffin-embedded human colon carcinoma using PPOX antibody (CAB6397) at dilution of 1:100 (40x lens). |
 | Immunofluorescence analysis of MCF7 cells using PPOX antibody (CAB6397). Blue: DAPI for nuclear staining. |
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