PPOX Antibody, Biotin conjugated

Code CSB-PA018502LD01HU
Size US$166
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Product Details

Full Product Name
Rabbit anti-Homo sapiens (Human) PPOX Polyclonal antibody
Uniprot No.
Target Names
PPOX
Alternative Names
MGC8485 antibody; PPO antibody; PPOX antibody; PPOX_HUMAN antibody; Protoporphyrinogen oxidase antibody; V290M antibody; Variegate porphyria antibody; VP antibody
Raised in
Rabbit
Species Reactivity
Human
Immunogen
Recombinant Human Protoporphyrinogen oxidase protein (1-477AA)
Immunogen Species
Homo sapiens (Human)
Conjugate
Biotin
Clonality
Polyclonal
Isotype
IgG
Purification Method
>95%, Protein G purified
Concentration
It differs from different batches. Please contact us to confirm it.
Buffer
Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
Form
Liquid
Tested Applications
ELISA
Protocols
Troubleshooting and FAQs
Storage
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time
Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

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Target Background

Function
Catalyzes the 6-electron oxidation of protoporphyrinogen-IX to form protoporphyrin-IX.
Gene References into Functions
  1. the regulation of PPOX gene expression can also occur through a post-transcriptional modulation of the amount of gene product and this modulation can be mediated by 5' untranslated exon 1. PMID: 27667166
  2. GAPDH and protoporphyrinogen oxidase were shown to have higher expression in faster growing cell lines and primary tumors. Pharmacologic inhibition of GAPDH or PPOX reduced the growth of colon cancer cells in vitro PMID: 25944804
  3. Variegate porphyria is the result of decreased protoporphyrinogen oxidase activity. Diet supplementation with vitamins E and C restores PPOX gene expression in lymphocytes of variegate porphyria patients. PMID: 23601071
  4. in the hepatic cancer tissue of two acute porphyria patients, somatic second-hit mutations result in nearly complete inactivation of PPOX and HMBS PMID: 25445397
  5. The c.851G>T and the c.1013C>G of PPOX gene were found in two and four unrelated families respectively. 1 experienced only photosensitivity, 1 only neurological symptoms and the 2 both clinical manifestations. PMID: 19656455
  6. The mutation 1082-1083insC(1 base pair insertion at position 1082) in exon 10 of the PPOX gene was prevalent in the Swiss population. PMID: 19656457
  7. findings emphasize the usefulness of MLPA analysis as a complement to PPOX gene sequencing analysis for comprehensive genetic diagnostics in patients with variegate prophyria PMID: 23324528
  8. VP-causing mutation affect the catalytic activity of hPPO by affecting the ability of hPPO to sample the privileged conformations PMID: 23467411
  9. sequenced 27 members of a family with variegate porphyria with a T>A transversion at the intron 6 consensus splicing site in 8 patients and 4 SNPs: c.-414A>C; IVS2+121G>C; c.1188G>A and IVS12+34C>T PMID: 19229653
  10. data deliver further confirmation that the South African and Dutch variegate porphyria families carrying mutation p.R59W shared a common ancestor. PMID: 21910705
  11. Forty-seven variegate porphyria-causing mutations were purified by chromatography and kinetically characterized in vitro. PMID: 21048046
  12. Two previously undescribed mutations were identified: PPOX1423-1426-delATCT and PPOX2272insG. PMID: 12017191
  13. This enzyme in its regular and mutagenized forms is targeted to mitochondria when transfected. PMID: 12556518
  14. Information required for targeting to the mitochondria is contained within the first 250 amino acid residues of human PPOX. PMID: 14535846
  15. Modulation of penetrance by the wild-type allele in dominantly inherited erythrohepatic and acute hepatic porphyrias was studied using PPOX. PMID: 14669009
  16. Protoporphyrinogen oxidase targeting mechanism to the mitochondrion. PMID: 16621625
  17. Mutation in protoporphyrinogen oxidase is associated with variegate porphyria PMID: 16947091
  18. GATA-1 binding sites in exon 1 constitute key regulatory elements in differential expression of PPOX in erythroid and non-erythroid cells. PMID: 18191920

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Involvement in disease
Variegate porphyria (VP)
Subcellular Location
Mitochondrion inner membrane; Peripheral membrane protein; Intermembrane side.
Protein Families
Protoporphyrinogen oxidase family
Tissue Specificity
Expressed in heart, brain, placenta, lung, liver, skeletal muscle, kidney and pancreas.
Database Links

HGNC: 9280

OMIM: 176200

KEGG: hsa:5498

STRING: 9606.ENSP00000343943

UniGene: Hs.517373

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