Gene Gene information from NCBI Gene database.
Entrez ID 83752
Gene name Lon peptidase 2, peroxisomal
Gene symbol LONP2
Synonyms (NCBI Gene)
LONPLONPLPLONPSLON
Chromosome 16
Chromosome location 16q12.1
Summary 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
miRNA miRNA information provided by mirtarbase database.
462
miRTarBase ID miRNA Experiments Reference
MIRT020862 hsa-miR-155-5p Proteomics 18668040
MIRT023972 hsa-miR-1-3p Proteomics 18668040
MIRT044448 hsa-miR-320a CLASH 23622248
MIRT041601 hsa-miR-193b-3p CLASH 23622248
MIRT038688 hsa-miR-30c-2-3p CLASH 23622248
Gene ontology (GO) Gene Ontology (GO) annotations describing the biological processes, molecular functions, and cellular components associated with a gene.
32
GO ID Ontology Definition Evidence Reference
GO:0000166 Function Nucleotide binding IEA
GO:0002020 Function Protease binding IPI 22002062
GO:0004176 Function ATP-dependent peptidase activity IEA
GO:0004252 Function Serine-type endopeptidase activity IEA
GO:0005515 Function Protein binding IPI 18281296, 27173435, 28514442, 32296183, 33961781
Other IDs Other IDs provides unique identifiers for this gene in OMIM, HGNC, and Ensembl databases.
MIM HGNC e!Ensembl
617774 20598 ENSG00000102910
Protein Protein information from UniProt database.
UniProt ID Unique identifier for the protein in the UniProt database. Click to view detailed protein information.
Q86WA8
Protein name Lon protease homolog 2, peroxisomal (EC 3.4.21.53) (Lon protease-like protein 2) (Lon protease 2) (Peroxisomal Lon protease) (pLon)
Protein function ATP-dependent serine protease that mediates the selective degradation of misfolded and unassembled polypeptides in the peroxisomal matrix. Necessary for type 2 peroxisome targeting signal (PTS2)-containing protein processing and facilitates pero
Family and domains

Pfam

Accession ID Position in sequence Description Type
PF02190 LON_substr_bdg 12 220 ATP-dependent protease La (LON) substrate-binding domain Family
PF00004 AAA 371 512 ATPase family associated with various cellular activities (AAA) Domain
PF05362 Lon_C 628 837 Lon protease (S16) C-terminal proteolytic domain Domain
Tissue specificity TISSUE SPECIFICITY: Widely expressed, with high levels in the liver, kidney and pancreas. {ECO:0000269|PubMed:18281296}.
Sequence
MSSVSPIQIPSRLPLLLTHEGVLLPGSTMRTSVDSARNLQLVRSRLLKGTSLQSTILGVI
PNTPDPASDAQDLPPLHRIGTAALAVQVVGSNWPKPHYTLLITGLCRFQIVQVLKEKPYP
IAEVEQLDRLEEFPNTCKMREELGELSEQFYKYAVQLVEMLDMSVPAVAKLRRLLDSLPR
EALPDILTSIIRTSNKEKLQILDAVSLEERFKMTIPLLVR
QIEGLKLLQKTRKPKQDDDK
RVIAIRPIRRITHISGTLEDEDEDEDNDDIVMLEKKIRTSSMPEQAHKVCVKEIKRLKKM
PQSMPEYALTRNYLELMVELPWNKSTTDRLDIRAARILLDNDHYAMEKLKKRVLEYLAVR
QLKNNLKGPILCFVGPPGVGKTSVGRSVAKTLGREFHRIALGGVCDQSDIRGHRRTYVGS
MPGRIINGLKTVGVNNPVFLLDEVDKLGKSLQGDPAAALLEVLDPEQNHNFTDHYLNVAF
DLSQVLFIATANTTATIPAALLDRMEIIQVPG
YTQEEKIEIAHRHLIPKQLEQHGLTPQQ
IQIPQVTTLDIITRYTREAGVRSLDRKLGAICRAVAVKVAEGQHKEAKLDRSDVTEREGC
REHILEDEKPESISDTTDLALPPEMPILIDFHALKDILGPPMYEMEVSQRLSQPGVAIGL
AWTPLGGEIMFVEASRMDGEGQLTLTGQLGDVMKESAHLAISWLRSNAKKYQLTNAFGSF
DLLDNTDIHLHFPAGAVTKDGPSAGVTIVTCLASLFSGRLVRSDVAMTGEITLRGLVLPV
GGIKDKVLAAHRAGLKQVIIPRRNEKDLEGIPGNVRQDLSFVTASCLDEVLNAAFDG
GFT
VKTRPGLLNSKL
Sequence length 852
Interactions View interactions
Pathways Pathway information has different metabolic/signaling pathways associated with genes.
  Reactome
    Peroxisomal protein import
Associated diseases Disease associations from ClinVar (causal & non-causal) and other databases (OMIM, Orphanet, GWAS, etc.).
3
Evidence Score: ★☆☆☆☆  Gene-disease association found in Text Mining only ★★☆☆☆  Found in Text Mining and Unknown/Other Associations ★★★☆☆  Reported in Unknown/Other Associations across ≥2 Sources ★★★★☆  ClinVar: Pathogenic/Likely Pathogenic (<5 Variants) ★★★★★  ClinVar: Pathogenic/Likely Pathogenic (≥5 Variants)
Unknown / Other Associations ClinVar entries with uncertain/conflicting evidence, and associations from other databases (OMIM, Orphanet, GWAS, etc.) where the gene is not established as causal.
Phenotype Name Clinical Significance Source Evidence Score
BURATTI-HAREL SYNDROME Disgenet
★★☆☆☆
Found in Text Mining + Unknown/Other Associations
NEURODEVELOPMENTAL DISORDERS Disgenet
★★☆☆☆
Found in Text Mining + Unknown/Other Associations
Prostate cancer Uncertain significance ClinVar
★★☆☆☆
Found in Text Mining + Unknown/Other Associations
Associations from Text Mining Disease associations identified through text mining
Disease Name Disease (Merged) Source PMID Relationship Type Evidence Score
Carcinoma Squamous Cell Squamous cell carcinoma Pubtator 35768804 Associate
★☆☆☆☆
Found in Text Mining only
cervical cancer Cervical Cancer BEFREE 29502128
★☆☆☆☆
Found in Text Mining only
Cervix carcinoma Cervix carcinoma BEFREE 29502128
★☆☆☆☆
Found in Text Mining only
Malignant Neoplasms Malignant Neoplasm BEFREE 29502128
★☆☆☆☆
Found in Text Mining only
Malignant tumor of cervix Cervical Tumor BEFREE 29502128
★☆☆☆☆
Found in Text Mining only
Osteoglophonic dwarfism Osteoglophonic dwarfism Pubtator 34589382 Stimulate
★☆☆☆☆
Found in Text Mining only
Urinary Bladder Neoplasms Urinary bladder neoplasms Pubtator 35358000 Associate
★☆☆☆☆
Found in Text Mining only