PPOX (protoporphyrinogen oxidase)

2016-01-01  

Identity

HGNC
LOCATION
1q23.3
LOCUSID
ALIAS
PPO,V290M,VP
FUSION GENES

Other Information

Locus ID:

NCBI: 5498
MIM: 600923
HGNC: 9280
Ensembl: ENSG00000143224

Variants:

dbSNP: 5498
ClinVar: 5498
TCGA: ENSG00000143224
COSMIC: PPOX

RNA/Proteins

Gene IDTranscript IDUniprot
ENSG00000143224ENST00000352210P50336
ENSG00000143224ENST00000367999P50336
ENSG00000143224ENST00000462866A0A1W2PQM0
ENSG00000143224ENST00000470607A0A1W2PRF9
ENSG00000143224ENST00000495483A0A1W2PNH4
ENSG00000143224ENST00000497522A0A1W2PPA5
ENSG00000143224ENST00000535223F5H825
ENSG00000143224ENST00000537523H0YFP3
ENSG00000143224ENST00000537829H0YFE1
ENSG00000143224ENST00000539753F5H1I5
ENSG00000143224ENST00000544598F5GZT7
ENSG00000143224ENST00000650741A0A494C1M8
ENSG00000143224ENST00000651150A0A494C0L4
ENSG00000143224ENST00000652100A0A494C0C8
ENSG00000143224ENST00000652103A0A494C0D4
ENSG00000143224ENST00000652182A0A494C146
ENSG00000143224ENST00000652297A0A494C134
ENSG00000143224ENST00000652473D3DVG2
ENSG00000143224ENST00000652729A0A494C0M9

Expression (GTEx)

0
5
10
15
20
25
30
35
40
45

Pathways

PathwaySourceExternal ID
Porphyrin and chlorophyll metabolismKEGGko00860
Porphyrin and chlorophyll metabolismKEGGhsa00860
Metabolic pathwaysKEGGhsa01100
MetabolismREACTOMER-HSA-1430728
Metabolism of porphyrinsREACTOMER-HSA-189445
Heme biosynthesisREACTOMER-HSA-189451

Protein levels (Protein atlas)

Not detected
Low
Medium
High

PharmGKB

Entity IDNameTypeEvidenceAssociationPKPDPMIDs
PA166190681givosiranChemicalLabelAnnotationassociated

References

Pubmed IDYearTitleCitations
349687942022Molecular characterization of a novel His333Arg variant of human protoporphyrinogen oxidase IX.0
349687942022Molecular characterization of a novel His333Arg variant of human protoporphyrinogen oxidase IX.0
338578412021The hydrogen bonding network involved Arg59 in human protoporphyrinogen IX oxidase is essential for enzyme activity.2
338578412021The hydrogen bonding network involved Arg59 in human protoporphyrinogen IX oxidase is essential for enzyme activity.2
322472862020Two new mutations in the PPOX gene in a patient with variegate porphyria.0
322472862020Two new mutations in the PPOX gene in a patient with variegate porphyria.0
303851472019Acute hepatic porphyrias: Identification of 46 hydroxymethylbilane synthase, 11 coproporphyrinogen oxidase, and 20 protoporphyrinogen oxidase novel mutations.2
303851472019Acute hepatic porphyrias: Identification of 46 hydroxymethylbilane synthase, 11 coproporphyrinogen oxidase, and 20 protoporphyrinogen oxidase novel mutations.2
295509082018An unusual diagnosis in a 31-year-old man with abdominal pain and hyponatremia.0
295509082018An unusual diagnosis in a 31-year-old man with abdominal pain and hyponatremia.0
276671662016The assessment of noncoding variant of PPOX gene in variegate porphyria reveals post-transcriptional role of the 5' untranslated exon 1.2
276671662016The assessment of noncoding variant of PPOX gene in variegate porphyria reveals post-transcriptional role of the 5' untranslated exon 1.2
254453972015Biallelic inactivation of protoporphyrinogen oxidase and hydroxymethylbilane synthase is associated with liver cancer in acute porphyrias.8
259448042015Highly Expressed Genes in Rapidly Proliferating Tumor Cells as New Targets for Colorectal Cancer Treatment.12
254453972015Biallelic inactivation of protoporphyrinogen oxidase and hydroxymethylbilane synthase is associated with liver cancer in acute porphyrias.8

Citation

Dessen P

PPOX (protoporphyrinogen oxidase)

Atlas Genet Cytogenet Oncol Haematol. 2016-01-01

Online version: http://atlasgeneticsoncology.org/gene/55572/ppox-(protoporphyrinogen-oxidase)