Target

Gene symbol :  
Lonp1
NCBI gene ID :  
74142
Gene full name :  
Lon peptidase 1, mitochondrial
Synonym :  
Prss15

Protein function

Protein name :  
Lon protease homolog, mitochondrial
ATP-dependent protease involved in mitochondrial protein quality control

Expected phenotype

Knockouts cause embryonic lethality; heterozygotes show mitochondrial dysfunction

Human disease relevance

Mutations cause CODAS syndrome and mitochondrial disorders

Model applications

Used in mitochondrial proteostasis and metabolic disease studies

Keywords:

Mitochondria, Protease, Protein quality control, CODAS syndrome

Availability & Delivery:

  • Cryo-recovery using in-house SOPF facilities
  • Worldwide delivery by professional breeders

Lonp1

conditional Knockout mouse

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Models with certified health status from professional breeders in US and Europe