UniProt ID | LONM_HUMAN | |
---|---|---|
UniProt AC | P36776 | |
Protein Name | Lon protease homolog, mitochondrial {ECO:0000255|HAMAP-Rule:MF_03120} | |
Gene Name | LONP1 {ECO:0000255|HAMAP-Rule:MF_03120} | |
Organism | Homo sapiens (Human). | |
Sequence Length | 959 | |
Subcellular Localization | Mitochondrion matrix . | |
Protein Description | 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. May also have a chaperone function in the assembly of inner membrane protein complexes. 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. [PubMed: 12198491] | |
Protein Sequence | MAASTGYVRLWGAARCWVLRRPMLAAAGGRVPTAAGAWLLRGQRTCDASPPWALWGRGPAIGGQWRGFWEASSRGGGAFSGGEDASEGGAEEGAGGAGGSAGAGEGPVITALTPMTIPDVFPHLPLIAITRNPVFPRFIKIIEVKNKKLVELLRRKVRLAQPYVGVFLKRDDSNESDVVESLDEIYHTGTFAQIHEMQDLGDKLRMIVMGHRRVHISRQLEVEPEEPEAENKHKPRRKSKRGKKEAEDELSARHPAELAMEPTPELPAEVLMVEVENVVHEDFQVTEEVKALTAEIVKTIRDIIALNPLYRESVLQMMQAGQRVVDNPIYLSDMGAALTGAESHELQDVLEETNIPKRLYKALSLLKKEFELSKLQQRLGREVEEKIKQTHRKYLLQEQLKIIKKELGLEKDDKDAIEEKFRERLKELVVPKHVMDVVDEELSKLGLLDNHSSEFNVTRNYLDWLTSIPWGKYSNENLDLARAQAVLEEDHYGMEDVKKRILEFIAVSQLRGSTQGKILCFYGPPGVGKTSIARSIARALNREYFRFSVGGMTDVAEIKGHRRTYVGAMPGKIIQCLKKTKTENPLILIDEVDKIGRGYQGDPSSALLELLDPEQNANFLDHYLDVPVDLSKVLFICTANVTDTIPEPLRDRMEMINVSGYVAQEKLAIAERYLVPQARALCGLDESKAKLSSDVLTLLIKQYCRESGVRNLQKQVEKVLRKSAYKIVSGEAESVEVTPENLQDFVGKPVFTVERMYDVTPPGVVMGLAWTAMGGSTLFVETSLRRPQDKDAKGDKDGSLEVTGQLGEVMKESARIAYTFARAFLMQHAPANDYLVTSHIHLHVPEGATPKDGPSAGCTIVTALLSLAMGRPVRQNLAMTGEVSLTGKILPVGGIKEKTIAAKRAGVTCIVLPAENKKDFYDLAAFITEGLEVHFVEHYREIFDIAFPDEQAEALAVER | |
Overview of Protein Modification Sites with Functional and Structural Information | ||
|
* ASA = Accessible Surface Area
Locations | Modification | Substrate Peptides & Secondary Structure |
ASA (%) | Reference | Orthologous Protein Cluster |
---|---|---|---|---|---|
4 | Phosphorylation | ----MAASTGYVRLW ----CCCCCCCHHHH | 16.90 | 20860994 | |
5 | Phosphorylation | ---MAASTGYVRLWG ---CCCCCCCHHHHH | 28.01 | 20860994 | |
7 | Phosphorylation | -MAASTGYVRLWGAA -CCCCCCCHHHHHCH | 5.18 | 20860994 | |
33 | Phosphorylation | AAGGRVPTAAGAWLL HCCCCCCCCCCHHHH | 26.70 | - | |
38 | Ubiquitination | VPTAAGAWLLRGQRT CCCCCCHHHHCCCCC | 8.60 | 24816145 | |
48 | Ubiquitination | RGQRTCDASPPWALW CCCCCCCCCCCEEEE | 26.53 | 24816145 | |
72 | Phosphorylation | WRGFWEASSRGGGAF CCCCHHCCCCCCCCC | 14.73 | 23312004 | |
73 | Phosphorylation | RGFWEASSRGGGAFS CCCHHCCCCCCCCCC | 41.83 | 23312004 | |
80 | Phosphorylation | SRGGGAFSGGEDASE CCCCCCCCCCCCCCC | 45.41 | 28348404 | |
145 | 2-Hydroxyisobutyrylation | FIKIIEVKNKKLVEL HHEEEEECCHHHHHH | 50.70 | - | |
145 | Succinylation | FIKIIEVKNKKLVEL HHEEEEECCHHHHHH | 50.70 | 23954790 | |
148 | Malonylation | IIEVKNKKLVELLRR EEEECCHHHHHHHHH | 68.13 | 26320211 | |
148 | Succinylation | IIEVKNKKLVELLRR EEEECCHHHHHHHHH | 68.13 | 27452117 | |
161 | Ubiquitination | RRKVRLAQPYVGVFL HHHHHHCCCEEEEEE | 34.15 | 24816145 | |
163 | Phosphorylation | KVRLAQPYVGVFLKR HHHHCCCEEEEEEEC | 9.58 | 28152594 | |
170 | Ubiquitination | YVGVFLKRDDSNESD EEEEEEECCCCCCCH | 55.93 | 24816145 | |
173 | Phosphorylation | VFLKRDDSNESDVVE EEEECCCCCCCHHHH | 46.95 | 28258704 | |
176 | Phosphorylation | KRDDSNESDVVESLD ECCCCCCCHHHHHHH | 39.70 | 28258704 | |
178 | Ubiquitination | DDSNESDVVESLDEI CCCCCCHHHHHHHHH | 7.77 | 29967540 | |
180 | Ubiquitination | SNESDVVESLDEIYH CCCCHHHHHHHHHHC | 45.49 | 24816145 | |
181 | Phosphorylation | NESDVVESLDEIYHT CCCHHHHHHHHHHCC | 29.52 | 26471730 | |
186 | Phosphorylation | VESLDEIYHTGTFAQ HHHHHHHHCCCCHHH | 7.41 | 22817900 | |
190 | Ubiquitination | DEIYHTGTFAQIHEM HHHHCCCCHHHHHHH | 19.56 | 24816145 | |
197 | Ubiquitination | TFAQIHEMQDLGDKL CHHHHHHHHHHHHHE | 2.02 | - | |
197 | Ubiquitination | TFAQIHEMQDLGDKL CHHHHHHHHHHHHHE | 2.02 | 29967540 | |
224 | Ubiquitination | SRQLEVEPEEPEAEN EEEEECCCCCCHHHC | 57.32 | 29967540 | |
234 | Ubiquitination | PEAENKHKPRRKSKR CHHHCCCCCCCCCCC | 41.66 | 24816145 | |
244 | Ubiquitination | RKSKRGKKEAEDELS CCCCCCCHHHHHHHH | 66.05 | 24816145 | |
293 | Ubiquitination | TEEVKALTAEIVKTI HHHHHHHHHHHHHHH | 27.40 | 24816145 | |
298 | 2-Hydroxyisobutyrylation | ALTAEIVKTIRDIIA HHHHHHHHHHHHHHH | 43.85 | - | |
302 | Ubiquitination | EIVKTIRDIIALNPL HHHHHHHHHHHHCHH | 32.56 | 24816145 | |
310 | Ubiquitination | IIALNPLYRESVLQM HHHHCHHHHHHHHHH | 17.37 | 29967540 | |
313 | Phosphorylation | LNPLYRESVLQMMQA HCHHHHHHHHHHHHC | 21.09 | 20860994 | |
317 | Sulfoxidation | YRESVLQMMQAGQRV HHHHHHHHHHCCCCC | 1.56 | 21406390 | |
322 | Ubiquitination | LQMMQAGQRVVDNPI HHHHHCCCCCCCCCE | 37.70 | 24816145 | |
329 | Ubiquitination | QRVVDNPIYLSDMGA CCCCCCCEEHHHHHH | 7.29 | 29967540 | |
356 | Ubiquitination | VLEETNIPKRLYKAL HHHHCCCCHHHHHHH | 20.13 | 29967540 | |
357 | Ubiquitination | LEETNIPKRLYKALS HHHCCCCHHHHHHHH | 51.76 | 24816145 | |
361 | Acetylation | NIPKRLYKALSLLKK CCCHHHHHHHHHHHH | 47.66 | 18586583 | |
364 | Phosphorylation | KRLYKALSLLKKEFE HHHHHHHHHHHHHHH | 36.19 | 24719451 | |
367 | 2-Hydroxyisobutyrylation | YKALSLLKKEFELSK HHHHHHHHHHHHHHH | 56.39 | - | |
368 | 2-Hydroxyisobutyrylation | KALSLLKKEFELSKL HHHHHHHHHHHHHHH | 68.55 | - | |
368 | Malonylation | KALSLLKKEFELSKL HHHHHHHHHHHHHHH | 68.55 | 26320211 | |
374 | Acetylation | KKEFELSKLQQRLGR HHHHHHHHHHHHHHH | 64.04 | 25953088 | |
374 | Succinylation | KKEFELSKLQQRLGR HHHHHHHHHHHHHHH | 64.04 | 27452117 | |
374 | Ubiquitination | KKEFELSKLQQRLGR HHHHHHHHHHHHHHH | 64.04 | 29967540 | |
383 | Ubiquitination | QQRLGREVEEKIKQT HHHHHHHHHHHHHHH | 12.71 | - | |
385 | Ubiquitination | RLGREVEEKIKQTHR HHHHHHHHHHHHHHH | 66.32 | 29967540 | |
386 | Ubiquitination | LGREVEEKIKQTHRK HHHHHHHHHHHHHHH | 41.52 | 24816145 | |
393 | Acetylation | KIKQTHRKYLLQEQL HHHHHHHHHHHHHHH | 32.42 | 27452117 | |
393 | Ubiquitination | KIKQTHRKYLLQEQL HHHHHHHHHHHHHHH | 32.42 | 29967540 | |
394 | Phosphorylation | IKQTHRKYLLQEQLK HHHHHHHHHHHHHHH | 16.77 | 22817900 | |
398 | Ubiquitination | HRKYLLQEQLKIIKK HHHHHHHHHHHHHHH | 58.83 | 29967540 | |
405 | 2-Hydroxyisobutyrylation | EQLKIIKKELGLEKD HHHHHHHHHHCCCCC | 48.00 | - | |
411 | Acetylation | KKELGLEKDDKDAIE HHHHCCCCCCHHHHH | 76.03 | 25953088 | |
414 | Acetylation | LGLEKDDKDAIEEKF HCCCCCCHHHHHHHH | 60.23 | 25953088 | |
420 | 2-Hydroxyisobutyrylation | DKDAIEEKFRERLKE CHHHHHHHHHHHHHH | 36.61 | - | |
420 | Acetylation | DKDAIEEKFRERLKE CHHHHHHHHHHHHHH | 36.61 | 26822725 | |
420 | Ubiquitination | DKDAIEEKFRERLKE CHHHHHHHHHHHHHH | 36.61 | 29967540 | |
426 | 2-Hydroxyisobutyrylation | EKFRERLKELVVPKH HHHHHHHHHCCCCHH | 56.05 | - | |
426 | Malonylation | EKFRERLKELVVPKH HHHHHHHHHCCCCHH | 56.05 | 26320211 | |
432 | 2-Hydroxyisobutyrylation | LKELVVPKHVMDVVD HHHCCCCHHHHHHHC | 36.90 | - | |
432 | Acetylation | LKELVVPKHVMDVVD HHHCCCCHHHHHHHC | 36.90 | 26822725 | |
434 | Ubiquitination | ELVVPKHVMDVVDEE HCCCCHHHHHHHCHH | 4.35 | 24816145 | |
435 | Sulfoxidation | LVVPKHVMDVVDEEL CCCCHHHHHHHCHHH | 3.02 | 21406390 | |
466 | Phosphorylation | RNYLDWLTSIPWGKY HHHHHHHHHCCCCCC | 21.98 | 20860994 | |
467 | Phosphorylation | NYLDWLTSIPWGKYS HHHHHHHHCCCCCCC | 25.20 | 20860994 | |
473 | Nitration | TSIPWGKYSNENLDL HHCCCCCCCCCCHHH | 16.88 | - | |
473 | Phosphorylation | TSIPWGKYSNENLDL HHCCCCCCCCCCHHH | 16.88 | 22210691 | |
474 | Phosphorylation | SIPWGKYSNENLDLA HCCCCCCCCCCHHHH | 40.95 | 22210691 | |
492 | Phosphorylation | AVLEEDHYGMEDVKK HHHHHCCCCHHHHHH | 30.41 | 20860994 | |
498 | 2-Hydroxyisobutyrylation | HYGMEDVKKRILEFI CCCHHHHHHHHHHHH | 48.97 | - | |
498 | Acetylation | HYGMEDVKKRILEFI CCCHHHHHHHHHHHH | 48.97 | 27452117 | |
498 | Ubiquitination | HYGMEDVKKRILEFI CCCHHHHHHHHHHHH | 48.97 | 24816145 | |
517 | Succinylation | LRGSTQGKILCFYGP HCCCCCCCEEEEECC | 23.76 | 27452117 | |
517 | Ubiquitination | LRGSTQGKILCFYGP HCCCCCCCEEEEECC | 23.76 | 29967540 | |
518 | Ubiquitination | RGSTQGKILCFYGPP CCCCCCCEEEEECCC | 5.32 | 29967540 | |
522 | Phosphorylation | QGKILCFYGPPGVGK CCCEEEEECCCCCCH | 27.94 | 28152594 | |
522 | Ubiquitination | QGKILCFYGPPGVGK CCCEEEEECCCCCCH | 27.94 | 24816145 | |
530 | Phosphorylation | GPPGVGKTSIARSIA CCCCCCHHHHHHHHH | 21.31 | 23186163 | |
530 | Ubiquitination | GPPGVGKTSIARSIA CCCCCCHHHHHHHHH | 21.31 | 29967540 | |
531 | Phosphorylation | PPGVGKTSIARSIAR CCCCCHHHHHHHHHH | 20.10 | 23186163 | |
548 | Phosphorylation | NREYFRFSVGGMTDV CCHHHCEECCCCCCH | 18.65 | 25850435 | |
552 | Acetylation | FRFSVGGMTDVAEIK HCEECCCCCCHHHHC | 2.08 | - | |
552 | Sulfoxidation | FRFSVGGMTDVAEIK HCEECCCCCCHHHHC | 2.08 | 21406390 | |
559 | 2-Hydroxyisobutyrylation | MTDVAEIKGHRRTYV CCCHHHHCCCCCCCC | 39.92 | - | |
564 | Phosphorylation | EIKGHRRTYVGAMPG HHCCCCCCCCCCCCC | 23.63 | 21406692 | |
565 | Phosphorylation | IKGHRRTYVGAMPGK HCCCCCCCCCCCCCH | 8.62 | 21406692 | |
578 | Acetylation | GKIIQCLKKTKTENP CHHHHHHHHCCCCCC | 67.05 | 25953088 | |
579 | Ubiquitination | KIIQCLKKTKTENPL HHHHHHHHCCCCCCE | 42.16 | - | |
580 | Phosphorylation | IIQCLKKTKTENPLI HHHHHHHCCCCCCEE | 41.43 | 23312004 | |
581 | 2-Hydroxyisobutyrylation | IQCLKKTKTENPLIL HHHHHHCCCCCCEEE | 63.82 | - | |
581 | Acetylation | IQCLKKTKTENPLIL HHHHHHCCCCCCEEE | 63.82 | 26051181 | |
581 | Ubiquitination | IQCLKKTKTENPLIL HHHHHHCCCCCCEEE | 63.82 | 29967540 | |
594 | 2-Hydroxyisobutyrylation | ILIDEVDKIGRGYQG EEEEECCCCCCCCCC | 52.84 | - | |
594 | Ubiquitination | ILIDEVDKIGRGYQG EEEEECCCCCCCCCC | 52.84 | 29967540 | |
600 | Ubiquitination | DKIGRGYQGDPSSAL CCCCCCCCCCCCHHH | 53.27 | 24816145 | |
605 | Phosphorylation | GYQGDPSSALLELLD CCCCCCCHHHHHHCC | 28.46 | 25338102 | |
637 | S-palmitoylation | LSKVLFICTANVTDT HHHEEEEEECCCCCC | 2.00 | 29575903 | |
650 | Ubiquitination | DTIPEPLRDRMEMIN CCCCHHHHHHHHHCC | 39.96 | 29967540 | |
654 | Ubiquitination | EPLRDRMEMINVSGY HHHHHHHHHCCCCHH | 37.49 | 24816145 | |
659 | Phosphorylation | RMEMINVSGYVAQEK HHHHCCCCHHHHHHH | 21.66 | 24144214 | |
661 | Phosphorylation | EMINVSGYVAQEKLA HHCCCCHHHHHHHHH | 5.77 | 24144214 | |
666 | 2-Hydroxyisobutyrylation | SGYVAQEKLAIAERY CHHHHHHHHHHHHHH | 31.01 | - | |
666 | Acetylation | SGYVAQEKLAIAERY CHHHHHHHHHHHHHH | 31.01 | 25953088 | |
692 | Phosphorylation | DESKAKLSSDVLTLL CHHHHHCCHHHHHHH | 24.64 | 20068231 | |
693 | Phosphorylation | ESKAKLSSDVLTLLI HHHHHCCHHHHHHHH | 42.02 | 21406692 | |
697 | Phosphorylation | KLSSDVLTLLIKQYC HCCHHHHHHHHHHHH | 21.26 | 21406692 | |
701 | Acetylation | DVLTLLIKQYCRESG HHHHHHHHHHHHHHC | 34.87 | 26051181 | |
714 | Succinylation | SGVRNLQKQVEKVLR HCCHHHHHHHHHHHH | 60.52 | 27452117 | |
714 | Ubiquitination | SGVRNLQKQVEKVLR HCCHHHHHHHHHHHH | 60.52 | 29967540 | |
718 | Succinylation | NLQKQVEKVLRKSAY HHHHHHHHHHHHHCH | 47.95 | 27452117 | |
718 | Ubiquitination | NLQKQVEKVLRKSAY HHHHHHHHHHHHHCH | 47.95 | 24816145 | |
732 | Ubiquitination | YKIVSGEAESVEVTP HHHHCCCCEEEEECC | 20.21 | 24816145 | |
748 | Acetylation | NLQDFVGKPVFTVER HHHHHCCCCCEEEEE | 32.48 | 25038526 | |
757 | Phosphorylation | VFTVERMYDVTPPGV CEEEEECCCCCCCCC | 17.30 | 21406692 | |
760 | Phosphorylation | VERMYDVTPPGVVMG EEECCCCCCCCCEEE | 22.37 | 21406692 | |
771 | Phosphorylation | VVMGLAWTAMGGSTL CEEEEEEEECCCCEE | 10.93 | 21406692 | |
776 | Phosphorylation | AWTAMGGSTLFVETS EEEECCCCEEEEEEC | 19.06 | 21406692 | |
777 | Phosphorylation | WTAMGGSTLFVETSL EEECCCCEEEEEECC | 27.73 | 21406692 | |
782 | Phosphorylation | GSTLFVETSLRRPQD CCEEEEEECCCCCCC | 27.97 | 21406692 | |
783 | Phosphorylation | STLFVETSLRRPQDK CEEEEEECCCCCCCC | 13.33 | 21406692 | |
796 | Ubiquitination | DKDAKGDKDGSLEVT CCCCCCCCCCCEEEE | 73.06 | 24816145 | |
799 | Phosphorylation | AKGDKDGSLEVTGQL CCCCCCCCEEEEEEH | 31.33 | 20068231 | |
803 | Phosphorylation | KDGSLEVTGQLGEVM CCCCEEEEEEHHHHH | 15.16 | 20068231 | |
810 | Sulfoxidation | TGQLGEVMKESARIA EEEHHHHHHHHHHHH | 3.29 | 21406390 | |
880 | Phosphorylation | VRQNLAMTGEVSLTG HHHHCEECCEEEECC | 25.28 | 20068231 | |
884 | Phosphorylation | LAMTGEVSLTGKILP CEECCEEEECCEEEE | 18.86 | 20068231 | |
886 | Phosphorylation | MTGEVSLTGKILPVG ECCEEEECCEEEEEC | 28.99 | 20068231 | |
904 | Methylation | EKTIAAKRAGVTCIV HHHHHHHHCCCEEEE | 31.67 | 115489235 | |
917 | Acetylation | IVLPAENKKDFYDLA EEEECCCCCCHHHHH | 45.27 | 25953088 | |
917 | Malonylation | IVLPAENKKDFYDLA EEEECCCCCCHHHHH | 45.27 | 26320211 |
Modified Location | Modified Residue | Modification | Type of Upstream Proteins | Gene Name of Upstream Proteins | UniProt AC of Upstream Proteins | Sources |
---|---|---|---|---|---|---|
Oops, there are no upstream regulatory protein records of LONM_HUMAN !! |
Modified Location | Modified Residue | Modification | Function | Reference | ||
---|---|---|---|---|---|---|
Oops, there are no descriptions of PTM sites of LONM_HUMAN !! |
* Distance = the distance between SAP position and PTM sites.
Modified Location | Modification | Variant Position (Distance <= 10) |
Residue Change | SAP | Related Disease | Reference |
---|---|---|---|---|---|---|
Oops, there are no SNP-PTM records of LONM_HUMAN !! |
Interacting Protein | Gene Name | Interaction Type | PPI Reference | Domain-Domain Interactions |
---|---|---|---|---|
CLPX_HUMAN | CLPX | physical | 26344197 |
Kegg Disease | ||||||
---|---|---|---|---|---|---|
There are no disease associations of PTM sites. | ||||||
OMIM Disease | ||||||
600373 | CODAS syndrome (CODASS) | |||||
Kegg Drug | ||||||
There are no disease associations of PTM sites. | ||||||
DrugBank | ||||||
There are no disease associations of PTM sites. |
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