LONP2

Protein identityUniProt · HPA

UniProt accession

Q86WA8

Gene

LONP2

Ensembl

ENSG00000102910

Chromosome

16

Canonical length

852 aa

Protein class

Enzymes, Predicted intracellular proteins

OverviewNCBI Gene

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 peroxisome homeostasis as well as proteolytically degrading peroxisomal proteins damaged by oxidation. The protein has an N-terminal Lon N substrate recognition domain, an ATPase domain, a proteolytic domain, and, in some isoforms, a C-terminal peroxisome targeting sequence. Alternative splicing results in multiple transcript variants encoding distinct isoforms. [provided by RefSeq, Jan 2017]

Canonical amino-acid sequenceUniProt

852 residues, UniProt reviewed canonical sequence.

>Q86WA8|LONP2
     1  MSSVSPIQIP SRLPLLLTHE GVLLPGSTMR TSVDSARNLQ LVRSRLLKGT SLQSTILGVI
    61  PNTPDPASDA QDLPPLHRIG TAALAVQVVG SNWPKPHYTL LITGLCRFQI VQVLKEKPYP
   121  IAEVEQLDRL EEFPNTCKMR EELGELSEQF YKYAVQLVEM LDMSVPAVAK LRRLLDSLPR
   181  EALPDILTSI IRTSNKEKLQ ILDAVSLEER FKMTIPLLVR QIEGLKLLQK TRKPKQDDDK
   241  RVIAIRPIRR ITHISGTLED EDEDEDNDDI VMLEKKIRTS SMPEQAHKVC VKEIKRLKKM
   301  PQSMPEYALT RNYLELMVEL PWNKSTTDRL DIRAARILLD NDHYAMEKLK KRVLEYLAVR
   361  QLKNNLKGPI LCFVGPPGVG KTSVGRSVAK TLGREFHRIA LGGVCDQSDI RGHRRTYVGS
   421  MPGRIINGLK TVGVNNPVFL LDEVDKLGKS LQGDPAAALL EVLDPEQNHN FTDHYLNVAF
   481  DLSQVLFIAT ANTTATIPAA LLDRMEIIQV PGYTQEEKIE IAHRHLIPKQ LEQHGLTPQQ
   541  IQIPQVTTLD IITRYTREAG VRSLDRKLGA ICRAVAVKVA EGQHKEAKLD RSDVTEREGC
   601  REHILEDEKP ESISDTTDLA LPPEMPILID FHALKDILGP PMYEMEVSQR LSQPGVAIGL
   661  AWTPLGGEIM FVEASRMDGE GQLTLTGQLG DVMKESAHLA ISWLRSNAKK YQLTNAFGSF
   721  DLLDNTDIHL HFPAGAVTKD GPSAGVTIVT CLASLFSGRL VRSDVAMTGE ITLRGLVLPV
   781  GGIKDKVLAA HRAGLKQVII PRRNEKDLEG IPGNVRQDLS FVTASCLDEV LNAAFDGGFT
   841  VKTRPGLLNS KL

LocalizationUniProt · AlphaFold · HPA

Whether an antibody against LONP2 can act on the native protein depends on physical access: surface and secreted proteins are reachable by circulating antibodies, intracellular proteins usually are not.

Antibody reachability

Intracellular

Secreted

No

Transmembrane segments

0

Mean surface accessibility (rSASA)

0.31

Highest tissue expression

81 nTPM

Expression across tissuesHPA

Genetic constraint and essentialitygnomAD · DepMap

Does the body need this protein intact? Low LOEUF or a strong DepMap dependency means loss or blockade of the protein is likely to be felt.

gnomAD LOEUF (loss-of-function intolerance)

1.35

gnomAD pLI

0

gnomAD missense Z

2.24

DepMap mean gene effect

0.07

DepMap dependency class

none

Cancer expressionTCGA

Across TCGA tumor cohorts, this protein is over-expressed in roughly 5% of surveyed tumor types (aggregate summary; per-cohort expression, alteration, and survival load in the interactive view).

OntologyGO

Biological processes

• peroxisome organization
• protein import into peroxisome matrix
• protein processing
• protein quality control for misfolded or incompletely synthesized proteins
• protein targeting to peroxisome
• regulation of fatty acid beta-oxidation

Molecular functions

• ATP binding
• ATP hydrolysis activity
• ATP-dependent peptidase activity
• enzyme binding
• peptidase activity
• protease binding
• serine-type endopeptidase activity

Cellular components

• cytosol
• membrane
• nucleus
• peroxisomal matrix
• peroxisome

Protein domainsUniProt · Pfam · InterPro

• Lon protease, N-terminal domain
• AAA+ ATPase domain
• ATPase, AAA-type, core
• Lon protease, bacterial/eukaryotic-type
• Peptidase S16, active site
• Peptidase S16, Lon proteolytic domain
• Small ribosomal subunit protein uS5 domain 2-type fold, subgroup
• PUA-like superfamily
• Ribosomal protein uS5 domain 2-type superfamily
• Lon protease
• P-loop containing nucleoside triphosphate hydrolase
• Lon protease, N-terminal domain superfamily
• Lon protease, AAA+ ATPase lid domain
• ATPase family associated with various cellular activities (AAA)
• ATP-dependent protease La (LON) substrate-binding domain
• Lon protease (S16) C-terminal proteolytic domain
• Lon protease AAA+ ATPase lid domain
• Lon protease homologue 2, peroxisomal

KeywordsUniProt

• Acetylation
• ATP-binding
• Hydrolase
• Nucleotide-binding
• Peroxisome
• Protease
• Serine protease

InteractionsUniProt · HPA

Protein binding partners of LONP2 in the human serome: UniProt-annotated complex subunits plus reported interactors. Each links to its own Seroatlas record.

• PEX5
• TYSND1
• ACOX1
• RSPH9