Ppox Gene Summary [Mouse]
Enables oxygen-dependent protoporphyrinogen oxidase activity. Involved in heme A biosynthetic process; heme B biosynthetic process; and heme O biosynthetic process. Acts upstream of or within heme biosynthetic process. Located in mitochondrial inner membrane. Is active in mitochondrial intermembrane space. Is expressed in several structures, including alimentary system; brain; genitourinary system; integumental system; and sensory organ. Used to study variegate porphyria. Human ortholog(s) of this gene implicated in variegate porphyria. Orthologous to human PPOX (protoporphyrinogen oxidase). [provided by Alliance of Genome Resources, Apr 2025]
Details
| Type | Retained Intron |
| Official Symbol | Ppox |
| Official Name | protoporphyrinogen oxidase [Source:MGI Symbol;Acc:MGI:104968] |
| Ensembl ID | ENSMUSG00000062729 |
| Bio databases IDs | |
| Aliases | protoporphyrinogen oxidase |
| Synonyms | PPO, protoporphyrinogen oxidase, V290M, VP, VPCO |
| Species | Mouse, Mus musculus |
| Orthologies |
Protein Domains
A protein domain is a distinct structural or functional region within a protein that can evolve, function, and exist independently of the rest of the protein chain. These domains in mouse Ppox often fold into stable, three-dimensional structures and are associated with specific biological functions, such as binding to DNA, other proteins, or small molecules.
- Rossmann-fold NAD(P)(+)-binding proteins
- protoporphyrinogen oxidase
- flavin adenine dinucleotide binding
- NAD(P)-binding Rossmann-like domain
- FAD dependent oxidoreductase
- enzyme
- protein binding
- Flavin containing amine oxidoreductase
Top Findings
The most significant associations for this gene, including commonly observed domains, pathway involvement, and functional highlights based on current data.
disease
- porphyria variegata
- childhood-onset variegate porphyria
- sickle cell anemia
role in cell
- expression in
- proliferation
- accumulation in
- respiration in
Subcellular Expression
Locations within the cell where the protein is known or predicted to be active, providing insight into its function and cellular context.
- Cytoplasm
- Mitochondria
- mitochondrial membrane
- mitochondrial intermembrane space
- mitochondrial inner membrane
Gene Ontology Annotations
Describes the biological processes, cellular components, and molecular functions associated with the mouse Ppox gene, providing context for its role in the cell.
Biological Process
Functions and activities the gene product is involved in
- heme O biosynthetic process
- porphyrin-containing compound biosynthetic process
- heme biosynthetic process
- heme a biosynthetic process
- protoporphyrinogen IX biosynthetic process
- heme b biosynthetic process
Cellular Component
Where in the cell the gene product is active
- mitochondrial intermembrane space
- mitochondrial membrane
- mitochondrial inner membrane
Molecular Function
What the gene product does at the molecular level
- flavin adenine dinucleotide binding
- oxygen-dependent protoporphyrinogen oxidase activity