Gene
lonp1
- ID
- ZDB-GENE-030131-4006
- Name
- lon peptidase 1, mitochondrial
- Symbol
- lonp1 Nomenclature History
- Previous Names
-
- fc64d11
- prss15 (1)
- wu:fc64d11
- Type
- protein_coding_gene
- Location
- Chr: 22 Mapping Details/Browsers
- Description
- Predicted to have ATP-dependent peptidase activity and single-stranded DNA binding activity. Predicted to be involved in chaperone-mediated protein complex assembly; mitochondrion organization; and protein quality control for misfolded or incompletely synthesized proteins. Predicted to localize to mitochondrial matrix. Human ortholog(s) of this gene implicated in CODAS syndrome. Orthologous to human LONP1 (lon peptidase 1, mitochondrial).
- Genome Resources
- Note
- None
- Comparative Information
-
- All Expression Data
- 3 figures from 2 publications
- Cross-Species Comparison
- High Throughput Data
- Thisse Expression Data
- No data available
Wild Type Expression Summary
- All Phenotype Data
- No data available
- Cross-Species Comparison
- Alliance
Phenotype Summary
Mutations
Human Disease
| Disease Ontology Term | Multi-Species Data | OMIM Term | OMIM Phenotype ID |
|---|---|---|---|
| CODAS syndrome | Alliance | CODAS syndrome | 600373 |
Domain, Family, and Site Summary
| Type | InterPro ID | Name |
|---|---|---|
| Active_site | IPR008268 | Peptidase S16, active site |
| Domain | IPR003111 | Lon protease, N-terminal domain |
| Domain | IPR003593 | AAA+ ATPase domain |
| Domain | IPR003959 | ATPase, AAA-type, core |
| Domain | IPR008269 | Peptidase S16, Lon proteolytic domain |
| Family | IPR004815 | Lon protease, bacterial/eukaryotic-type |
| Family | IPR027065 | Lon protease |
| Family | IPR027503 | Lon protease homologue, chloroplastic/mitochondrial |
| Homologous_superfamily | IPR014721 | Small ribosomal subunit protein uS5 domain 2-type fold, subgroup |
| Homologous_superfamily | IPR015947 | PUA-like superfamily |
| Homologous_superfamily | IPR020568 | Ribosomal protein uS5 domain 2-type superfamily |
| Homologous_superfamily | IPR027417 | P-loop containing nucleoside triphosphate hydrolase |
| Homologous_superfamily | IPR046336 | Lon protease, N-terminal domain superfamily |
Domain Details Per Protein
| Protein | Length | AAA+ ATPase domain | ATPase, AAA-type, core | Lon protease | Lon protease, bacterial/eukaryotic-type | Lon protease homologue, chloroplastic/mitochondrial | Lon protease, N-terminal domain | Lon protease, N-terminal domain superfamily | Peptidase S16, active site | Peptidase S16, Lon proteolytic domain | P-loop containing nucleoside triphosphate hydrolase | PUA-like superfamily | Ribosomal protein uS5 domain 2-type superfamily | Small ribosomal subunit protein uS5 domain 2-type fold, subgroup |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
|
UniProtKB:A0A0R4IH79
|
966 |
Interactions and Pathways
No data available
Plasmids
No data available