Lonp1
entrez
170916
canonical
uniprot
Q924S5
canonical
ensembl
ENSRNOG00000046502
resolver
ensembl
ENSRNOG00055015957
resolver
ensembl
ENSRNOG00060017387
resolver
ensembl
ENSRNOG00065013729
resolver
ensp
ENSRNOP00000066618
resolver
ensp
ENSRNOP00055021877
resolver
ensp
ENSRNOP00060023928
resolver
ensp
ENSRNOP00065017526
resolver
enst
ENSRNOT00055027137
resolver
enst
ENSRNOT00060029711
resolver
enst
ENSRNOT00065022588
resolver
genesymbol
Lonp1
resolver
genesymbol_syn
Lon
resolver
genesymbol_syn
Prss15
resolver
uniprot_entry
LONM_RAT
resolver
amino_acid_sequence
MAASTGYVRLWAAARCWVLRRPLLAVTGGRVPSASGSWLRRGCRVCDTSTPWGGRVPMGGGQWRGLWDAGSRGGSDETSEGGVEDGATASSGEGPVVTALAPMTVPDVFPHLPLIAISRNPVFPRFIKIVEVKNKKLVELLRRKVRLAQPYVGVFLKRDDNNESDVVESLDEIYHTGTFAQIHEMQDLGDKLRMIVTGHRRIHISRQLEVEPEGLEPEAENKQKSRRKLKRGKKEVGDELGAKPQLEMVTEATSDTSKEVLMVEVENVAHEDFQVTEEVKALTAEIVKTIRDIIALNPLYRESVLQMMQAGQRVVDNPIYLSDMGAALTGAESHELQDVLEETNILKRLYKALSLLKKEFELSKLQQRLGREVEEKIKQTHRKYLLQEQLKIIKKELGLEKDDKDAIEEKFRERLKELVVPKHVMDVVDEELSKLALLDNHSSEFNVTRNYLDWLTSIPWGRQSDENLDLARAQSVLEEDHYGMEDVKKRVLEFIAVSQLRGSTQGKILCFHGPPGVGKTSIARSIARALGREYFRFSVGGMTDVAEIKGHRRTYVGAMPGKIIQCLKKTKTENPLVLIDEVDKIGRGYQGDPSSALLELLDPEQNANFLDHYLDVPVDLSKVLFICTANVTDTIPEPLRDRMEMINVSGYVAQEKLAIAERYLVPQARTLCGLDESKAQLSATVLTLLIKQYCRESGVRNLQKQVEKVLRKAAYKIVSGEAQTVHVTPENLQDFVGKPVFTVERMYDVTPPGVVMGLAWTAMGGSTLFVETSLRRPQPSGSKEDKDGSLEVTGQLGDVMKESARIAYTFARAFLMEQDPENDFLVTSHIHLHVPEGATPKDGPSAGCTIVTALLSLALGQPVLQNLAMTGEVSLTGKVLPVGGIKEKTIAAKRAGVTCIILPAENRKDFSDLAPFITEGLEVHFVEHYRDIFRIAFPLREHQEALAVER
uniprot
proteins
ec_number
3.4.21.53
uniprot
proteins
ec_number
3.6.4.10
uniprot
proteins
function
FUNCTION: ATP-dependent serine protease that mediates the selective degradation of misfolded, unassembled or oxidatively damaged polypeptides as well as certain short-lived regulatory proteins in the mitochondrial matrix. Endogenous substrates include mitochondrial steroidogenic acute regulatory (StAR) protein, DELE1, helicase Twinkle (TWNK) and the large ribosomal subunit protein MRPL32/bL32m. MRPL32/bL32m is protected from degradation by LONP1 when it is bound to a nucleic acid (RNA), but TWNK is not. In addtion to its protease activity, has also an ATP-dependent protein folding chaperone activity and functions in mitochondrial protein folding. Participates in the regulation of mitochondrial gene expression and in the maintenance of the integrity of the mitochondrial genome. Binds to mitochondrial promoters and RNA in a single-stranded, site-specific, and strand-specific manner. May regulate mitochondrial DNA replication and/or gene expression using site-specific, single-stranded DNA binding to target the degradation of regulatory proteins binding to adjacent sites in mitochondrial promoters. {ECO:0000250|UniProtKB:P36776, ECO:0000255|HAMAP-Rule:MF_03120, ECO:0000269|PubMed:12752449, ECO:0000269|PubMed:15560797}.
uniprot
proteins
mass_dalton
105793
uniprot
proteins
post_translational_modification
PTM: Phosphorylated. Phosphorylation at Tyr-174 and Tyr-384 inhibits the ATP-dependent protease activity. {ECO:0000250|UniProtKB:P36776}.
uniprot
proteins
protein_family
Peptidase S16 family
uniprot
proteins
pubmed
12082077
uniprot
proteins
pubmed
12752449
uniprot
proteins
pubmed
15560797
uniprot
proteins
pubmed
7961901
uniprot
proteins
sequence_length
950
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: Mitochondrion matrix {ECO:0000255|HAMAP-Rule:MF_03120, ECO:0000269|PubMed:12752449, ECO:0000269|PubMed:15560797, ECO:0000269|PubMed:7961901}.
uniprot
proteins