Lonp1
entrez
74142
canonical
uniprot
Q8CGK3
canonical
ensembl
ENSMUSG00000041168
resolver
ensp
ENSMUSP00000041814
resolver
enst
ENSMUST00000047226
resolver
genesymbol
Lonp1
resolver
genesymbol_syn
Prss15
resolver
uniprot
Q3TSK9
resolver
uniprot
Q3TVL2
resolver
uniprot
Q3TXE4
resolver
uniprot
Q3TZW3
resolver
uniprot
Q9DBP9
resolver
uniprot_entry
LONM_MOUSE
resolver
uniprot_sec
Q3TSK9
resolver
uniprot_sec
Q3TVL2
resolver
uniprot_sec
Q3TXE4
resolver
uniprot_sec
Q3TZW3
resolver
uniprot_sec
Q9DBP9
resolver
amino_acid_sequence
MAASTGYVRLWAAARCWVLRRPLLAVTGGRVPSASGSWLRRGCRACDMSAPWGGRVLPGGVQWRGLWDSGNRGGSDETSEGGAEDGATASTGEGPVVTALAPMTVPDVFPHLPLIAITRNPVFPRFIKIVEVKNKKLVELLRRKVRLAQPYVGVFLKRDDNNESDVVESLDEIYHTGTFAQIHEMQDLGDKLRMIVTGHRRIHISRQLEVEPEGLEPEAEKQKSRRKLKRGKKEVEDELGPKPQLEMVTEAATDTSKEVLMVEVENVAHEDFQVTEEVKALTAEIVKTIRDIIALNPLYRESVLQMMQAGQRVVDNPIYLSDMGAALTGAESHELQDVLEETNILKRLYKALSLLKKEFELSKLQQRLGREVEEKIKQTHRKYLLQEQLKIIKKELGLEKDDKDAIEEKFRERLRELVVPKHVMDVVDEELSKLALLDNHSSEFNVTRNYLDWLTSIPWGRQSDENLDLARAQAVLEEDHYGMEDVKKRVLEFIAVSQLRGSTQGKILCFHGPPGVGKTSIARSIARALGREYFRFSVGGMTDVAEIKGHRRTYVGAMPGKIIQCLKKTKTENPLVLIDEVDKIGRGYQGDPSSALLELLDPEQNANFLDHYLDVPVDLSKVLFICTANVIDTIPEPLRDRMEMINVSGYVAQEKLAIAERYLVPQARTLCGLDESKAQLSAAVLTLLIKQYCRESGVRNLQKQVEKVLRKAAYKIVSGEAQTVQVTPENLQDFVGKPVFTVERMYEVTPPGVVMGLAWTAMGGSTLFVETSLRRPQPSGSKEDKDGSLEVTGQLGDVMKESARIAYTYARAFLMEQDPENDFLVTSHIHLHVPEGATPKDGPSAGCTIVTALLSLALGQPVLQNLAMTGEVSLTGKVLPVGGIKEKTIAAKRAGVTCIILPAENRKDYSDLAPFITEGLEVHFVEHYRDIFPIAFPRREHREALAVER
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. 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:12657466}.
uniprot
proteins
mass_dalton
105843
uniprot
proteins
post_translational_modification
PTM: Phosphorylated. Phosphorylation at Tyr-174 and Tyr-383 inhibits the ATP-dependent protease activity. {ECO:0000250|UniProtKB:P36776}.
uniprot
proteins
protein_family
Peptidase S16 family
uniprot
proteins
pubmed
12657466
uniprot
proteins
pubmed
16141072
uniprot
proteins
pubmed
21183079
uniprot
proteins
sequence_length
949
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: Mitochondrion matrix {ECO:0000255|HAMAP-Rule:MF_03120, ECO:0000269|PubMed:12657466}.
uniprot
proteins