MIPEP Gene Summary [Human]
The product of this gene performs the final step in processing a specific class of nuclear-encoded proteins targeted to the mitochondrial matrix or inner membrane. This protein is primarily involved in the maturation of oxidative phosphorylation (OXPHOS)-related proteins. This gene may contribute to the functional effects of frataxin deficiency and the clinical manifestations of Friedreich ataxia. [provided by RefSeq, Jul 2008]
Details
| Type | Processed Transcript |
| Official Symbol | MIPEP |
| Official Name | mitochondrial intermediate peptidase [Source:HGNC Symbol;Acc:HGNC:7104] |
| Ensembl ID | ENSG00000027001 |
| Bio databases IDs | |
| Aliases | mitochondrial intermediate peptidase |
| Synonyms | 5730405E07Rik, COXPD31, HMIP, LOC100505145, MIP, Mitochondrial Intermediate Peptidase |
| Species | Human, Homo sapiens |
| 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 human MIPEP often fold into stable, three-dimensional structures and are associated with specific biological functions, such as binding to DNA, other proteins, or small molecules.
- endopeptidase
- peptidase
- protein binding
- Peptidase family M3
- peptidase GluZincin family
- mitochondrial intermediate peptidase
- metalloendopeptidase
Top Findings
The most significant associations for this gene, including commonly observed domains, pathway involvement, and functional highlights based on current data.
disease
- combined oxidative phosphorylation deficiency 31
- hereditary disorder
- prostatic intraepithelial neoplasm
- prostatic intraepithelial neoplasia
- venous thromboembolism
- rheumatoid arthritis
- mitochondrial disorder
- prostate cancer
- pathologic myopia
regulates
role in cell
- proliferation
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 matrix
Gene Ontology Annotations
Describes the biological processes, cellular components, and molecular functions associated with the human MIPEP gene, providing context for its role in the cell.
Biological Process
Functions and activities the gene product is involved in
- protein processing involved in protein targeting to mitochondrion
- peptide metabolic process
Cellular Component
Where in the cell the gene product is active
- mitochondrial matrix
- mitochondrion
Molecular Function
What the gene product does at the molecular level
- metal ion binding
- metalloendopeptidase activity