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Lonp1 Gene Detail
Summary
  • Symbol
    Lonp1
  • Name
    lon peptidase 1, mitochondrial
  • Synonyms
    1200017E13Rik, LON, Prss15
  • Feature Type
    protein coding gene
  • IDs
    MGI:1921392
    NCBI Gene: 74142
  • Gene Overview
    MyGene.info: LONP1
Location & Maps
more
  • Sequence Map
    Chr17:56614301-56626903 bp, - strand
  • From Ensembl annotation of GRCm38

    Mouse Genome Browser

  • Download
    Sequence
      12603 bp   ±  kb flank

  • Genome Browsers
  • Genetic Map
    Chromosome 17, 29.42 cM, cytoband D
  • Mapping Data
    2 experiments
Homology
more
  • Human Ortholog
    LONP1, lon peptidase 1, mitochondrial
  • Vertebrate Orthologs
    10
  • Human Ortholog
    LONP1, lon peptidase 1, mitochondrial
    Orthology source: HomoloGene, HGNC
  • Synonyms
    CODASS, hLON, LON, LonHS, LONP, PIM1, PRSS15
  • Links
    NCBI Gene ID: 9361
    neXtProt AC: NX_P36776

  • Chr Location
    19p13.2; chr19:5691834-5720452 (-)  GRCh38.p2

Human Diseases
more
  • Diseases
    1 with human LONP1 associations

Human Disease Mouse Models
       CODAS Syndrome   OMIM: 600373
Click on a disease name to see all genes associated with that disease.

Mutations,
Alleles, and
Phenotypes
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  • Phenotype Summary
    7 phenotypes from 1 allele in 1 genetic background
    7 phenotype references
Phenotype Overview

adipose tissue
behavior/neurological
cardiovascular system
cellular
craniofacial
digestive/alimentary system
embryo
endocrine/exocrine glands
growth/size/body
hearing/vestibular/ear
hematopoietic system
homeostasis/metabolism
integument
immune system
limbs/digits/tail
liver/biliary system
mortality/aging
muscle
nervous system
pigmentation
renal/urinary system
reproductive system
respiratory system
skeleton
taste/olfaction
neoplasm
vision/eye

Click cells to view annotations.
Mice homozygous for a knock-out allele exhibit embryonic lethality with embryonic growth retardation, small size and decreased mitochondrial DNA content. Mice heterozygous for this allele exhibit reduced chemically-induced tumors.
Gene Ontology
(GO)
Classifications
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  • All GO Annotations
  • GO References
Molecular Function

carbohydrate derivative binding
cytoskeletal protein binding
DNA binding
enzyme regulator
hydrolase
ligase
lipid binding
oxidoreductase
receptor
receptor binding
RNA binding
transcription
transferase
transporter
Biological Process

nucleic acid-templated transcription
carbohydrate derivative metabolism
cell death
cell differentiation
cell proliferation
cellular component organization
establishment of localization
homeostatic process
immune system process
lipid metabolic process
protein metabolic process
response to stimulus
signaling
system development
Cellular Component

cell projection
cytoplasmic vesicle
cytoskeleton
cytosol
endoplasmic reticulum
endosome
extracellular region
Golgi apparatus
mitochondrion
non-membrane-bounded organelle
nucleus
organelle envelope
organelle lumen
plasma membrane
synapse
vacuole
Click cells to view annotations.
Expression
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Expression Overview

early conceptus
embryo ectoderm
embryo endoderm
embryo mesoderm
embryo mesenchyme
extraembryonic component
alimentary system
auditory system
branchial arches
cardiovascular system
connective tissue
endocrine system
exocrine system
hemolymphoid system
integumental system
limbs
liver and biliary system
musculoskeletal system
nervous system
olfactory system
reproductive system
respiratory system
urinary system
visual system
Click cells to view annotations.


  • Assay Results
  • Tissues
  • cDNA Data
  • Literature Summary
Interactions
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Sequences &
Gene Models
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Representative SequencesLengthStrain/SpeciesFlank
genomic ENSMUSG00000041168 Ensembl Gene Model | MGI Sequence Detail 12603 C57BL/6J ±  kb
transcript ENSMUST00000047226 Ensembl | MGI Sequence Detail 2963 Not Applicable  
polypeptide ENSMUSP00000041814 Ensembl | MGI Sequence Detail 949 Not Applicable  
For the selected sequence
Polymorphisms
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  • SNPs within 2kb
    181 from dbSNP Build 142
Protein
Information
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  • UniProt
    3 Sequences
  • Protein Ontology
    PR:000009880 Lon protease, mitochondrial
  • EC
  • InterPro Domains
    IPR003593 AAA+ ATPase domain
    IPR003959 ATPase, AAA-type, core
    IPR003111 ATP-dependent protease La (LON), substrate-binding domain
    IPR027065 Lon protease
    IPR004815 Lon protease, bacterial/eukaryotic-type
    IPR027503 Lon protease homolog, chloroplastic/mitochondrial
    IPR008268 Peptidase S16, active site
    IPR008269 Peptidase S16, Lon C-terminal
    IPR027417 P-loop containing nucleoside triphosphate hydrolase
    IPR015947 PUA-like domain
    IPR020568 Ribosomal protein S5 domain 2-type fold
    IPR014721 Ribosomal protein S5 domain 2-type fold, subgroup
Molecular
Reagents
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  • All nucleic 143
    cDNA 142
    Other 1

    Microarray probesets 4
References
more
  • Summaries
    All 36
    Developmental Gene Expression 2
    Gene Ontology 8
    Phenotypes 7
  • Earliest
    J:65060 Kawai J, et al., Functional annotation of a full-length mouse cDNA collection. Nature. 2001 Feb 8;409(6821):685-90
  • Latest
    J:221992 Quiros PM, et al., ATP-dependent Lon protease controls tumor bioenergetics by reprogramming mitochondrial activity. Cell Rep. 2014 Jul 24;8(2):542-56

Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
Citing These Resources
Funding Information
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last database update
09/27/2016
MGI 6.05
The Jackson Laboratory