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MGP002387

Record overview

MGPD IDMGP002387
Gene ID5498
SpeciesHomo sapiens (Human)
Gene Nameprotoporphyrinogen oxidase
Gene Symbol PPOX
SynonymsVP; PPO; V290M;
Alternate namesprotoporphyrinogen oxidase;
Chromosome1
Map Location1q22
EC Number1.3.3.4;
SummaryThis 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]
OrthologsView orthologs and multiple alignments for PPOX

Proteins

protoporphyrinogen oxidase
Refseq ID:NP_001116236
Protein GI:170650615
UniProt ID:P50336
mRNA ID:NM_001122764
Length:477
RefSeq Status:
MGRTVVVLGGGISGLAASYHLSRAPCPPKVVLVESSERLGGWIRSVRGPNGAIFELGPRGIRPAGALGARTLLLVSELGLDSEVLPVRGDHPAAQNRFLY
VGGALHALPTGLRGLLRPSPPFSKPLFWAGLRELTKPRGKEPDETVHSFAQRRLGPEVASLAMDSLCRGVFAGNSRELSIRSCFPSLFQAEQTHRSILLG
LLLGAGRTPQPDSALIRQALAERWSQWSLRGGLEMLPQALETHLTSRGVSVLRGQPVCGLSLQAEGRWKVSLRDSSLEADHVISAIPASVLSELLPAEAA
PLARALSAITAVSVAVVNLQYQGAHLPVQGFGHLVPSSEDPGVLGIVYDSVAFPEQDGSPPGLRVTVMLGGSWLQTLEASGCVLSQELFQQRAQEAAATQ
LGLKEMPSHCLVHLHKNCIPQYTLGHWQKLESARQFLTAHRLPLTLAGASYEGVAVNDCIESGRQAAVSVLGTEPNS
 
protoporphyrinogen oxidase
Refseq ID:NP_000300
Protein GI:4506001
UniProt ID:P50336
mRNA ID:NM_000309
Length:477
RefSeq Status:
Protein sequence is identical to GI:170650615 (mRNA isoform)
 
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