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PPOX

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]
Full Name
protoporphyrinogen oxidase
Function
Catalyzes the 6-electron oxidation of protoporphyrinogen-IX to form protoporphyrin-IX.
Biological Process
Heme biosynthetic processManual Assertion Based On ExperimentIDA:UniProtKB
Porphyrin-containing compound biosynthetic processManual Assertion Based On ExperimentIDA:UniProtKB
Protoporphyrinogen IX biosynthetic processIEA:UniProtKB-UniPathway
Cellular Location
Mitochondrion inner membrane
Involvement in disease
Variegate porphyria (VP):
A form of porphyria. 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. Variegate porphyria 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.
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Anti-PPOX antibodies

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Mouse Anti-PPOX Recombinant Antibody (2B5) (CBMAB-A6954-LY)

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Target: PPOX
Host: Mouse
Antibody Isotype: IgG2a, κ
Specificity: Human
Clone: 2B5
Application*: WB, E
Datasheet SDS

Mouse Anti-PPOX Recombinant Antibody (1G2) (CBMAB-A6953-LY)

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Target: PPOX
Host: Mouse
Antibody Isotype: IgG2a, κ
Specificity: Human
Clone: 1G2
Application*: WB, E
Datasheet SDS

Mouse Anti-PPOX Recombinant Antibody (1E1) (CBMAB-A6952-LY)

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Target: PPOX
Host: Mouse
Antibody Isotype: IgG1, κ
Specificity: Human
Clone: 1E1
Application*: WB, E
Datasheet SDS

Mouse Anti-PPOX Recombinant Antibody (CBYC-P561) (CBMAB-P2579-YC)

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Target: PPOX
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: CBYC-P561
Application*: E, IH, WB
Datasheet SDS

Rabbit Anti-PPOX Recombinant Antibody (CBYC-P559) (CBMAB-P2577-YC)

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Target: PPOX
Host: Rabbit
Antibody Isotype: IgG
Specificity: Human, Mouse, Rat
Clone: CBYC-P559
Application*: WB, F, IC, IF
Datasheet SDS

Mouse Anti-PPOX Recombinant Antibody (2F10) (CBMAB-P2576-YC)

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Target: PPOX
Host: Mouse
Antibody Isotype: IgG1, κ
Specificity: Human
Clone: 2F10
Application*: E, P, RNAi, WB
Datasheet SDS
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(P): Predicted
* Abbreviations
  • AActivation
  • AGAgonist
  • APApoptosis
  • BBlocking
  • BABioassay
  • BIBioimaging
  • CImmunohistochemistry-Frozen Sections
  • CIChromatin Immunoprecipitation
  • CTCytotoxicity
  • CSCostimulation
  • DDepletion
  • DBDot Blot
  • EELISA
  • ECELISA(Cap)
  • EDELISA(Det)
  • ESELISpot
  • EMElectron Microscopy
  • FFlow Cytometry
  • FNFunction Assay
  • GSGel Supershift
  • IInhibition
  • IAEnzyme Immunoassay
  • ICImmunocytochemistry
  • IDImmunodiffusion
  • IEImmunoelectrophoresis
  • IFImmunofluorescence
  • IHImmunohistochemistry
  • IMImmunomicroscopy
  • IOImmunoassay
  • IPImmunoprecipitation
  • ISIntracellular Staining for Flow Cytometry
  • LALuminex Assay
  • LFLateral Flow Immunoassay
  • MMicroarray
  • MCMass Cytometry/CyTOF
  • MDMeDIP
  • MSElectrophoretic Mobility Shift Assay
  • NNeutralization
  • PImmunohistologyp-Paraffin Sections
  • PAPeptide Array
  • PEPeptide ELISA
  • PLProximity Ligation Assay
  • RRadioimmunoassay
  • SStimulation
  • SESandwich ELISA
  • SHIn situ hybridization
  • TCTissue Culture
  • WBWestern Blot
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