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 enable 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 act upstream of or within protein catabolic process and proteolysis. Predicted to be located in mitochondrion. Predicted to be active in mitochondrial matrix. Is expressed in midbrain hindbrain boundary. Human ortholog(s) of this gene implicated in CODAS syndrome. Orthologous to human LONP1 (lon peptidase 1, mitochondrial).
Genome Resources
Note
None
Comparative Information
Expression
All Expression Data
1 figure from Kim et al., 2021
Cross-Species Comparison
High Throughput Data
Thisse Expression Data
No data available
Wild Type Expression Summary
Phenotype
All Phenotype Data
No data available
Cross-Species Comparison
Alliance
Phenotype Summary
Mutations
Mutants
Sequence Targeting Reagents
Human Disease
Associated With lonp1 Human Ortholog
Disease Ontology Term Multi-Species Data OMIM Term OMIM Phenotype ID
CODAS syndrome Alliance CODAS syndrome 600373
Associated With lonp1 Via Experimental Models
No data available
Gene Ontology
Protein Domains
Domain, Family, and Site Summary
Type InterPro ID Name
Active_site IPR008268 Peptidase S16, active site
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 Ribosomal protein S5 domain 2-type fold, subgroup
Homologous_superfamily IPR015947 PUA-like superfamily
Homologous_superfamily IPR020568 Ribosomal protein S5 domain 2-type fold
Homologous_superfamily IPR027417 P-loop containing nucleoside triphosphate hydrolase
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 Peptidase S16, active site Peptidase S16, Lon proteolytic domain P-loop containing nucleoside triphosphate hydrolase PUA-like superfamily Ribosomal protein S5 domain 2-type fold Ribosomal protein S5 domain 2-type fold, subgroup
UniProtKB:A0A0R4IPW4 247
UniProtKB:A0A0R4IH79 966
Transcripts
Genome Browsers
Interactions and Pathways
No data available
Antibodies
No data available
Plasmids
No data available
Constructs
Marker Relationships
Sequences
Orthology
Comparative Orthology
Alliance
Gene Tree
Ensembl
Citations