• Gene id: 83752

Gene Summary

• Gene Symbol: LONP2   Gene   UCSC   Ensembl
• Aliases: LONP, LONPL, PLON, PSLON
• Gene name: lon peptidase 2, peroxisomal
• Alternate names: lon protease homolog 2, peroxisomal, lon protease 2, lon protease-like protein 2, peroxisomal LON protease like, peroxisomal Lon protease homolog 2,
• Gene location: 16q12.1 (125520201: 125446643)     Exons: 15     NC_000003.12
• Gene summary(Entrez): In human, peroxisomes function primarily to catalyze fatty acid beta-oxidation and, as a by-product, produce hydrogen peroxide and superoxide. The protein encoded by this gene is an ATP-dependent protease that likely plays a role in maintaining overall pe
• OMIM: 617774

Protein Summary

• Protein general information: Q86WA8  
  • Name: Lon protease homolog 2, peroxisomal (EC 3.4.21.53) (Lon protease like protein 2) (Lon protease 2) (Peroxisomal Lon protease) (pLon)
  • Length: 852  
  • Mass: 94617
  • Tissue specificity: Widely expressed, with high levels in the liver, kidney and pancreas. {ECO
• Sequence:

  MSSVSPIQIPSRLPLLLTHEGVLLPGSTMRTSVDSARNLQLVRSRLLKGTSLQSTILGVIPNTPDPASDAQDLPP
  LHRIGTAALAVQVVGSNWPKPHYTLLITGLCRFQIVQVLKEKPYPIAEVEQLDRLEEFPNTCKMREELGELSEQF
  YKYAVQLVEMLDMSVPAVAKLRRLLDSLPREALPDILTSIIRTSNKEKLQILDAVSLEERFKMTIPLLVRQIEGL
  KLLQKTRKPKQDDDKRVIAIRPIRRITHISGTLEDEDEDEDNDDIVMLEKKIRTSSMPEQAHKVCVKEIKRLKKM
  PQSMPEYALTRNYLELMVELPWNKSTTDRLDIRAARILLDNDHYAMEKLKKRVLEYLAVRQLKNNLKGPILCFVG
  PPGVGKTSVGRSVAKTLGREFHRIALGGVCDQSDIRGHRRTYVGSMPGRIINGLKTVGVNNPVFLLDEVDKLGKS
  LQGDPAAALLEVLDPEQNHNFTDHYLNVAFDLSQVLFIATANTTATIPAALLDRMEIIQVPGYTQEEKIEIAHRH
  LIPKQLEQHGLTPQQIQIPQVTTLDIITRYTREAGVRSLDRKLGAICRAVAVKVAEGQHKEAKLDRSDVTEREGC
  REHILEDEKPESISDTTDLALPPEMPILIDFHALKDILGPPMYEMEVSQRLSQPGVAIGLAWTPLGGEIMFVEAS
  RMDGEGQLTLTGQLGDVMKESAHLAISWLRSNAKKYQLTNAFGSFDLLDNTDIHLHFPAGAVTKDGPSAGVTIVT
  CLASLFSGRLVRSDVAMTGEITLRGLVLPVGGIKDKVLAAHRAGLKQVIIPRRNEKDLEGIPGNVRQDLSFVTAS
  CLDEVLNAAFDGGFTVKTRPGLLNSKL

• Structural information:
  • Protein Domains: (13..22-)
    (/note="Lon-N-terminal)
    (/evidence="ECO:0000255|PROSITE-ProRule:PRU01123-)
    (651..83-)
    (/note="Lon-proteolytic)
    (/evidence="ECO:0000255|PROSITE-ProRule:PRU01122"-)
  • Interpro: IPR003593  IPR003959  IPR004815  IPR008269  IPR027065   IPR003111  IPR027501  IPR027417  IPR008268  IPR015947  IPR020568  IPR014721  
  • Prosite: PS51787 PS51786 PS01046
  • STRING: ENSP00000285737
• Other Databases: GeneCards:  LONP2  Malacards:  LONP2

Gene ontology

GO accession	Term name	Evidence code	Go category
GO:0005777	peroxisome	IDA	cellular component
GO:0016485	protein processing	IBA	biological process
GO:0006625	protein targeting to peroxisome	IBA	biological process
GO:0005782	peroxisomal matrix	IBA	cellular component
GO:0004252	serine-type endopeptidase activity	IEA	molecular function
GO:0006508	proteolysis	IEA	biological process
GO:0004176	ATP-dependent peptidase activity	IEA	molecular function
GO:0005524	ATP binding	IEA	molecular function
GO:0006515	protein quality control for misfolded or incompletely synthesized proteins	IEA	biological process
GO:0030163	protein catabolic process	IEA	biological process
GO:0016787	hydrolase activity	IEA	molecular function
GO:0006508	proteolysis	IEA	biological process
GO:0008233	peptidase activity	IEA	molecular function
GO:0005524	ATP binding	IEA	molecular function
GO:0008236	serine-type peptidase activity	IEA	molecular function
GO:0000166	nucleotide binding	IEA	molecular function
GO:0005777	peroxisome	IEA	cellular component
GO:0005782	peroxisomal matrix	TAS	cellular component
GO:0005829	cytosol	TAS	cellular component
GO:0005829	cytosol	TAS	cellular component
GO:0006625	protein targeting to peroxisome	TAS	biological process
GO:0005515	protein binding	IPI	molecular function
GO:0005515	protein binding	IPI	molecular function
GO:0005515	protein binding	IPI	molecular function
GO:0014070	response to organic cyclic compound	IEA	biological process
GO:0005777	peroxisome	IEA	cellular component
GO:0005782	peroxisomal matrix	IEA	cellular component
GO:0005634	nucleus	IEA	cellular component
GO:0005737	cytoplasm	IEA	cellular component
GO:0005782	peroxisomal matrix	IEA	cellular component
GO:0004252	serine-type endopeptidase activity	IEA	molecular function
GO:0016485	protein processing	IEA	biological process
GO:0005782	peroxisomal matrix	IEA	cellular component
GO:0004176	ATP-dependent peptidase activity	IEA	molecular function
GO:0005524	ATP binding	IEA	molecular function
GO:0006515	protein quality control for misfolded or incompletely synthesized proteins	IEA	biological process
GO:0016887	ATPase activity	IEA	molecular function
GO:0016558	protein import into peroxisome matrix	IEA	biological process
GO:0005777	peroxisome	IDA	cellular component
GO:0005777	peroxisome	IDA	cellular component
GO:0008233	peptidase activity	IDA	molecular function
GO:0016020	membrane	HDA	cellular component
GO:0016485	protein processing	IMP	biological process
GO:0006625	protein targeting to peroxisome	IMP	biological process
GO:0031998	regulation of fatty acid beta-oxidation	IMP	biological process
GO:0019899	enzyme binding	IPI	molecular function
GO:0005515	protein binding	IPI	molecular function
GO:0002020	protease binding	IPI	molecular function
GO:0007031	peroxisome organization	NAS	biological process

Diseases

Associated diseases	References
Male factor infertility	MIK: 29961538
Teratozoospermia	MIK: 17327269

PubMed references

PMID	Condition	Mutation	Ethnicity	Population details	Infertility_type	Associated_genes
17327269	Teratozoospermia			13 (5 controls, 8 cases)	Male infertility	GSE6967 analyzed by GEO2R (cutoff 1.5 fold)
29961538	Male factor infertility			318 (128 couples presenting with OAT (MF) and 118 maternal age-matched control (no MF) subjects undergoing infertility treatment, 72 surplus cryopreserved blastocysts)	Male infertility	RNA-seq