KEGG   ORTHOLOGY: K08675
Entry
K08675                      KO                                     
Symbol
PRSS15, PIM1
Name
ATP-dependent Lon protease [EC:3.4.21.53]
Disease
H01824  CODAS syndrome
Brite
KEGG Orthology (KO) [BR:ko00001]
 09180 Brite Hierarchies
  09181 Protein families: metabolism
   01002 Peptidases and inhibitors
    K08675  PRSS15, PIM1; ATP-dependent Lon protease
  09182 Protein families: genetic information processing
   03029 Mitochondrial biogenesis
    K08675  PRSS15, PIM1; ATP-dependent Lon protease
Enzymes [BR:ko01000]
 3. Hydrolases
  3.4  Acting on peptide bonds (peptidases)
   3.4.21  Serine endopeptidases
    3.4.21.53  endopeptidase La
     K08675  PRSS15, PIM1; ATP-dependent Lon protease
Peptidases and inhibitors [BR:ko01002]
 Serine peptidases
  Family S16: lon protease family
   K08675  PRSS15, PIM1; ATP-dependent Lon protease
Mitochondrial biogenesis [BR:ko03029]
 Mitochondrial quality control factors
  Regulator of mitochondrial biogenesis
   Other regulator of mitochondrial biogenesis
    K08675  PRSS15, PIM1; ATP-dependent Lon protease
Genes
HSA: 9361(LONP1)
PTR: 455623(LONP1)
PPS: 100976253(LONP1)
PON: 100461951(LONP1)
NLE: 100590941(LONP1)
HMH: 116483302(LONP1)
MCC: 700284(LONP1)
MCF: 101866482(LONP1)
MTHB: 126942484
MNI: 105484910(LONP1)
CSAB: 103233746(LONP1)
CATY: 105591837(LONP1)
PANU: 101020782(LONP1)
TGE: 112611827(LONP1)
MLEU: 105530113(LONP1)
RRO: 104665476(LONP1)
RBB: 108512096(LONP1)
TFN: 117076243(LONP1)
PTEH: 111522002(LONP1)
CANG: 105512862(LONP1)
CJC: 100408135(LONP1)
SBQ: 101042739(LONP1)
CIMI: 108304139(LONP1)
CSYR: 103252100(LONP1)
MMUR: 105869644(LONP1)
LCAT: 123636531(LONP1)
PCOQ: 105811918(LONP1)
OGA: 100946855(LONP1)
MMU: 74142(Lonp1)
MCAL: 110284091(Lonp1)
MPAH: 110335615(Lonp1)
RNO: 170916(Lonp1)
MCOC: 116074348(Lonp1)
ANU: 117705103(Lonp1)
MUN: 110546250(Lonp1)
CGE: 100771385(Lonp1)
MAUA: 101824017(Lonp1)
PROB: 127216770(Lonp1)
PLEU: 114685086(Lonp1)
MORG: 121459773(Lonp1)
MFOT: 126490356
AAMP: 119801143(Lonp1)
NGI: 103737205(Lonp1)
HGL: 101710908(Lonp1)
CPOC: 100717869(Lonp1)
CCAN: 109688221(Lonp1)
DORD: 105990295(Lonp1)
DSP: 122122771(Lonp1)
PLOP: 125348575(Lonp1)
NCAR: 124968663
MMMA: 107157309(Lonp1)
OPI: 101517458 101524431(LONP1)
TUP: 102469312(LONP1)
GVR: 103590755(LONP1)
CFA: 611425(LONP1)
CLUD: 112666341(LONP1)
VVP: 112935610(LONP1)
VLG: 121494821(LONP1)
NPO: 129522539(LONP1)
AML: 100468447(LONP1)
UMR: 103681856(LONP1)
UAH: 113242858(LONP1)
UAR: 123778997(LONP1)
ELK: 111161696
LLV: 125088026
MPUF: 101677927(LONP1)
MNP: 132010837(LONP1)
MLK: 131823353(LONP1)
NVS: 122909837(LONP1)
ORO: 101367221(LONP1)
EJU: 114226282(LONP1)
ZCA: 113917844(LONP1)
MLX: 118004987(LONP1)
NSU: 110593780(LONP1)
LWW: 102735352(LONP1)
FCA: 101093417(LONP1)
PYU: 121021334(LONP1)
PCOO: 112852855(LONP1)
PBG: 122486758(LONP1)
PVIV: 125152926(LONP1)
LRUF: 124523873
PPAD: 109255924(LONP1)
PUC: 125928896
AJU: 106975449
HHV: 120242036(LONP1)
BTA: 510796(LONP1)
BOM: 102273928(LONP1)
BIU: 109561716(LONP1)
BBUB: 102413839(LONP1)
BBIS: 104979909(LONP1)
CHX: 102182026(LONP1)
OAS: 101109965(LONP1)
BTAX: 128051425(LONP1)
ODA: 120864855(LONP1)
CCAD: 122440267(LONP1)
MBEZ: 129558820(LONP1)
SSC: 100511917(LONP1)
CFR: 102505491(LONP1)
CBAI: 105078242(LONP1)
CDK: 105091759(LONP1)
VPC: 102528772(LONP1)
BACU: 103008682(LONP1)
BMUS: 118891959(LONP1)
LVE: 103076731(LONP1)
OOR: 101275103(LONP1)
DLE: 111165960(LONP1)
PCAD: 102980645(LONP1)
PSIU: 116750480(LONP1)
NASI: 112400142(LONP1)
ECB: 100065045(LONP1)
EAI: 106832338(LONP1)
MYB: 102247241(LONP1)
MYD: 102751426(LONP1)
MMYO: 118658803(LONP1)
MLF: 102419649(LONP1)
PKL: 118731171(LONP1)
EFUS: 103294202(LONP1)
MNA: 107525668(LONP1)
DRO: 112312463(LONP1)
SHON: 118984540(LONP1)
AJM: 119063750(LONP1)
PDIC: 114502593(LONP1)
PHAS: 123813802(LONP1)
MMF: 118644009(LONP1)
PPAM: 129061544(LONP1)
HAI: 109388581(LONP1)
RFQ: 117037628(LONP1)
PALE: 102877695(LONP1)
PGIG: 120615422(LONP1)
PVP: 105301098(LONP1)
RAY: 107510942(LONP1)
MJV: 108404619(LONP1)
TOD: 119240611(LONP1)
SARA: 101550633(LONP1)
SETR: 126029737(LONP1)
LAV: 100664035(LONP1)
TMU: 101354513
ETF: 101645576(LONP1)
DNM: 101442081(LONP1)
MDO: 100024990(LONP1)
GAS: 123242749(LONP1)
SHR: 100926021(LONP1)
PCW: 110197391(LONP1)
OAA: 100074993(LONP1)
GGA: 768942(LONP1)
PCOC: 116236204(LONP1)
MGP: 100538450(LONP1)
CJO: 107325567(LONP1)
TPAI: 128090088(LONP1)
LMUT: 125684833(LONP1)
NMEL: 110388910(LONP1)
APLA: 101789952(LONP1)
ACYG: 106045695(LONP1)
CATA: 118258982(LONP1)
AFUL: 116499076(LONP1)
TGU: 100221382
PMOA: 120496370(LONP1)
OTC: 121345765(LONP1)
PRUF: 121354653(LONP1)
OMA: 130264474(LONP1)
CBRC: 103620665(LONP1)
ETL: 114065555(LONP1)
ZAB: 102070914
ACHL: 103798694(LONP1)
SVG: 106857968(LONP1)
MMEA: 130579786(LONP1)
HRT: 120763410(LONP1)
CLV: 102084316(LONP1)
NNI: 104022911(LONP1)
PCRI: 104034829(LONP1)
PCAO: 104046893(LONP1)
TALA: 104356016(LONP1)
PADL: 103916053(LONP1)
AFOR: 103903102(LONP1)
ACHC: 115348939(LONP1)
HALD: 104310957(LONP1)
HLE: 104840009(LONP1)
AGEN: 126041787
GCL: 127025662
CCRI: 104162974(LONP1)
CSTI: 104560528(LONP1)
CMAC: 104485312(LONP1)
MUI: 104539892(LONP1)
BREG: 104641260(LONP1)
FGA: 104074773(LONP1)
LDI: 104354414(LONP1)
MNB: 103770147(LONP1)
OHA: 104330522(LONP1)
NNT: 104400016(LONP1)
SHAB: 115618236(LONP1)
DPUB: 104308565(LONP1)
PGUU: 104466131(LONP1)
ACAR: 104521864(LONP1)
CPEA: 104397314(LONP1)
AVIT: 104268333(LONP1)
CVF: 104282491(LONP1)
RTD: 128900625(LONP1)
CUCA: 104064650(LONP1)
TEO: 104382132(LONP1)
BRHI: 104498183(LONP1)
AROW: 112967593(LONP1)
NPD: 112943938(LONP1)
TGT: 104567533(LONP1)
DNE: 112986235(LONP1)
SCAM: 104142034(LONP1)
AMJ: 102570078(LONP1)
CPOO: 109320518(LONP1)
GGN: 109294748(LONP1)
PSS: 102456397(LONP1)
CMY: 102939356(LONP1)
CCAY: 125627017(LONP1)
DCC: 119848230(LONP1)
CPIC: 101938534(LONP1)
TST: 117869052(LONP1)
CABI: 116822591(LONP1)
MRV: 120391769(LONP1)
ASAO: 132780973(LONP1)
PVT: 110072570(LONP1)
SUND: 121917762(LONP1)
PBI: 103066216(LONP1)
CTIG: 120305995(LONP1)
TSR: 106546171(LONP1)
PGUT: 117677422(LONP1)
APRI: 131188178(LONP1)
PTEX: 113435202(LONP1)
NSS: 113414185(LONP1)
VKO: 123025168(LONP1)
PMUA: 114588237(LONP1)
PRAF: 128405511(LONP1)
ZVI: 118085908(LONP1)
HCG: 128343155(LONP1)
GJA: 107111284(LONP1)
STOW: 125432716(LONP1)
EMC: 129331222(LONP1)
XLA: 108712696(lonp1.L)
XTR: 100492505(lonp1)
NPR: 108789637(LONP1)
RTEM: 120915321(LONP1)
BBUF: 120989567(LONP1)
BGAR: 122946317(LONP1)
MUO: 115480732(LONP1)
GSH: 117364918(LONP1)
DRE: 563257(lonp1)
SRX: 107744642
SGH: 107548847
CCAR: 109060510
CAUA: 113064659(lonp1)
CGIB: 127942691
PTET: 122327764(lonp1)
LROH: 127153900(lonp1)
OMC: 131530968(lonp1)
PPRM: 120486206(lonp1)
RKG: 130073582(lonp1)
MAMB: 125263194(lonp1)
CIDE: 127521893
TROS: 130548410(lonp1) 130548563
TDW: 130429594(lonp1)
MANU: 129453719(lonp1)
IPU: 100304917
IFU: 128613478(lonp1)
PHYP: 113543618(lonp1)
SMEO: 124391039(lonp1)
TFD: 113650940(lonp1)
TVC: 132853884(lonp1)
AMEX: 103026089(lonp1)
CMAO: 118818869(lonp1)
EEE: 113591724(lonp1)
CHAR: 105901004(lonp1)
TRU: 101061296(lonp1)
TFS: 130528636(lonp1)
LCO: 104927279(lonp1)
TBEN: 117490974(lonp1)
CGOB: 115007216(lonp1)
PGEO: 117445641(lonp1)
GACU: 117558678(lonp1)
EMAC: 134870058(lonp1)
ELY: 117254349(lonp1)
EFO: 125896154(lonp1)
PLEP: 121941221(lonp1)
SLUC: 116058827(lonp1)
ECRA: 117949957(lonp1)
ESP: 116695188(lonp1)
PFLV: 114561737(lonp1)
GAT: 120823493(lonp1)
PPUG: 119216691(lonp1)
AFB: 129089398(lonp1)
CLUM: 117729887(lonp1)
PSWI: 130193590(lonp1)
SCHU: 122877788(lonp1)
CUD: 121512834(lonp1)
ALAT: 119028929(lonp1)
MZE: 101475120(lonp1)
ONL: 100708000(lonp1)
OAU: 116320813(lonp1)
OLA: 101175387(lonp1)
OML: 112136791(lonp1)
CSAI: 133457992(lonp1)
XMA: 102219683(lonp1)
XCO: 114151434(lonp1)
XHE: 116716724(lonp1)
PRET: 103464218(lonp1)
PFOR: 103130498(lonp1)
PLAI: 106938571(lonp1)
PMEI: 106907085(lonp1) 106912100
GAF: 122838376(lonp1)
PPRL: 129354658(lonp1)
CVG: 107094448(lonp1)
CTUL: 119776304(lonp1)
GMU: 124873999(lonp1)
NFU: 107394993(lonp1)
KMR: 108246953(lonp1)
ALIM: 106517295(lonp1)
NWH: 119407910(lonp1)
AOCE: 111564042(lonp1)
MCEP: 125009504(lonp1)
CSEM: 103396817(lonp1)
POV: 109625362(lonp1)
SSEN: 122781173(lonp1)
HHIP: 117760370(lonp1)
HSP: 118121088(lonp1)
SMAU: 118318420(lonp1)
LCF: 108900694
SDU: 111240094(lonp1)
SLAL: 111660174(lonp1)
XGL: 120791102(lonp1)
HCQ: 109516958(lonp1)
SSCV: 125969085
SBIA: 133503829(lonp1)
PEE: 133406086(lonp1)
PTAO: 133480725(lonp1)
BPEC: 110162805(lonp1)
MALB: 109972174
BSPL: 114854160(lonp1)
SJO: 128361331(lonp1)
SASA: 106560597(lonp1)
STRU: 115158824(lonp1)
OTW: 112251435(lonp1)
OMY: 110524835(lonp1)
OGO: 123996570(lonp1)
ONE: 115108695(lonp1)
OKI: 109879504(lonp1)
OKE: 118394253(lonp1)
SALP: 111975733
SNH: 120055076(lonp1)
CCLU: 121534126(lonp1) 121540395
ELS: 105031384(lonp1)
SFM: 108921380(lonp1)
PKI: 111847564(lonp1)
AANG: 118224913(lonp1)
LOC: 102689553(lonp1)
PSPA: 121301396(lonp1)
PSEX: 120537829(lonp1)
LCM: 102359473(LONP1)
CMK: 103188337(lonp1)
RTP: 109915921(lonp1)
CPLA: 122565258(lonp1)
HOC: 132828792(lonp1)
LERI: 129711025(lonp1)
PMRN: 116953619(LONP1)
LRJ: 133355053(LONP1)
BFO: 118410223
BBEL: 109471798
CIN: 100178941
SCLV: 120347415
SPU: 100889553
LPIC: 129262353
AJC: 117108799
SKO: 100369052
DME: Dmel_CG8798(Lon)
DER: 6545346
DSE: 6619377
DSI: Dsimw501_GD12266(Dsim_GD12266)
DAN: 6493589
DSR: 110189879
DWI: 6643034
DGR: 6558113
DNV: 108650987
DHE: 111605069
DVI: 6622872
CCAT: 101450474
BOD: 106616907
BDR: 105229440
RZE: 108367300
AOQ: 129240247
SCAC: 106086277
LCQ: 111677879
LSQ: 119605668
GFS: 119645384
ECOE: 129942908
HIS: 119648820
AGA: 1272588
ACOZ: 120960116
AARA: 120899698
AMER: 121598536
ASTE: 118504878
AFUN: 125769965
AMOU: 128300687
AALI: 118463399
AAG: 5568011
AALB: 109399481
CPII: 120416668
CNS: 116339417
AME: 409459
ACER: 107994137
ALAB: 122718675
ADR: 102672731
AFLR: 100871499
BIM: 100748909
BBIF: 117213734
BVK: 117243522
BVAN: 117163289
BTER: 100645793
BAFF: 126915191
BPYO: 122568438
BPAS: 132909517
FVI: 122538931
CCAL: 108628100
OBB: 114882760
OLG: 117611743
MGEN: 117217357
NMEA: 116434103
CGIG: 122402756
SOC: 105205478
MPHA: 105833796
AEC: 105147324
ACEP: 105624556
PBAR: 105434097
VEM: 105570504
HST: 105187595
DQU: 106746169
CFO: 105251874
FEX: 115236366
LHU: 105673088
PGC: 109851632
OBO: 105275464
PCF: 106784053
PFUC: 122519145
VPS: 122630478
VCRB: 124424111
VVE: 124950506
NVI: 100119946
CSOL: 105364819
TPRE: 106648587
LHT: 122500227
LBD: 127290389
MDL: 103577847
CGLO: 123258423
FAS: 105267800
DAM: 107037927
AGIF: 122851396
CINS: 118065397
VCAN: 122411818
CCIN: 107270703
DSM: 124411534
NPT: 124220493
NFB: 124183771
NLO: 107217946
NVG: 124306273
AROA: 105689400
TCA: 661788
DPA: 109540187
SOY: 115882875
AGRG: 126741638
ATD: 109599976
CSET: 123314977
AGB: 108907705
LDC: 111508177
NVL: 108560099
PPYR: 116166586
OTU: 111413910
BMOR: 101738203
MSEX: 115444768
BANY: 112050155
MJU: 123874284
NIQ: 126773201
VCD: 124534572
MCIX: 123659190
PPOT: 106104836
PXU: 106122521
PRAP: 111003238
PBX: 123706790
PNAP: 125050092
ZCE: 119839171
CCRC: 123698568
AAGE: 121734390
HAW: 110378874
HZE: 124636789
TNL: 113493795
SLIU: 111354919
OFU: 114364564
PXY: 105395669
PGW: 126370846
CFEL: 113369819
CCRN: 123298023
API: 100162246
DNX: 107169899
AGS: 114125525
RMD: 113550433
DVT: 126903151
BTAB: 109041197
DCI: 103506165
CLEC: 106665553
HHAL: 106685637
NLU: 111051123
HVI: 124365758
FOC: 113217030
TPAL: 117648537
ZNE: 110840355
CSEC: 111874993
SGRE: 126266639
BROR: 134543433
IEL: 124163093
DMK: 116921792
DPZ: 124327004
PVM: 113813288
PCHN: 125039520
PMOO: 119586529
HAME: 121871465
PCLA: 123767327
CQD: 128698915
PTRU: 123518158
ESN: 126997515
MNZ: 135199524
HAZT: 108674639
EAF: 111715911
LSM: 121115751
TCF: 131886857
DSV: 119442891
RSAN: 119388011
RMP: 119187116
VDE: 111252124
VJA: 111269282
DPTE: 113798312
DFR: 124493312
PTEP: 107455378
ABRU: 129968512
UDV: 129221453
SDM: 118193105
LPOL: 106471332
PMEO: 129597571
CEL: CELE_C34B2.6(lonp-1) CELE_Y75B8A.4(lonp-2)
BMY: BM_BM13929(Bma-lonp-1)
PVUL: 126817890
BGT: 106071143
GAE: 121384968
HRF: 124145063
HRJ: 124257588
CRG: 105344337
CVN: 111131342
CANU: 128178221
OED: 125650151
MYI: 110451944
PMAX: 117334290
MCAF: 127734753
DPOL: 127878175
OBI: 106872622
OSN: 115231161
NVE: 5502549
EPA: 110243112
ATEN: 116297464
PDAM: 113682626
SPIS: 111342707
DGT: 114533888
XEN: 124433271
HMG: 100213990
HSY: 130645368
ATH: AT3G05780(LON3) AT3G05790(LON4) AT5G26860(LON1)
THJ: 104820100
CPAP: 110815328
CIT: 102620332
PVY: 116106956
TCC: 18600571
EGR: 104428250
VRA: 106772262
VAR: 108325921
VUN: 114191692
VUM: 124826734
CCAJ: 109819213
APRC: 113871088
MTR: 11410058
TPRA: 123890361
CAM: 101510578
PSAT: 127091504
LJA: Lj0g3v0183909.1(Lj0g3v0183909.1)
ADU: 107465214
AIP: 107618105
PCIN: 129316325
FVE: 101313904
RCN: 112192813
PPER: 18774588
PMUM: 103344549
PAVI: 110762891
ZJU: 107407834
CSAV: 115719194
CSV: 101204375
CMO: 103499387
BHJ: 120080427
MCHA: 111023269
CMAX: 111465425
CMOS: 111444355
CPEP: 111798716
JCU: 105647420
MESC: 110621016
JRE: 108981232
CILL: 122315884
CAVE: 132171266
QSU: 112028757
TWL: 120008555
VVI: 100254508
VRI: 117930785
SLY: 101264562
SPEN: 107002475
SOT: 102590267
SSTN: 125864712
CANN: 107845715
LBB: 132621092
NTA: 107796400
NSY: 104228685
NTO: 104120154
NAU: 109233012
ITR: 116032498
SIND: 105176824
OEU: 111390212
EGT: 105954679
SMIL: 130995406
SHIS: 125205924
APAN: 127260981
HAN: 110918283
ECAD: 122596376
LSV: 111878015
CCAV: 112516489
DCR: 108209611
CSIN: 114288218
RVL: 131325618
BVG: 104890770
SOE: 110796705
ATRI: 130809147
MOF: 131154546
PSOM: 113297859
BDI: 100844749
ATS: 109738385
TUA: 125535550
LPER: 127331411
SBI: 8077511
SITA: 101775616
SVS: 117842958
PHAI: 112880666
EGU: 105039934
MUS: 103990534
DCT: 110099479
PEQ: 110036410
AOF: 109842711
MSIN: 131224812
NCOL: 116254827
ATR: 18430231
PPP: 112290438
APRO: F751_5552
MPP: MICPUCDRAFT_33463(JGI:MicpuC2_33463)
SCE: YBL022C(PIM1)
SEUB: DI49_0159
ERC: Ecym_6190
KMX: KLMA_20035(PIM1)
NCS: NCAS_0B05620(NCAS0B05620)
NDI: NDAI_0B02960(NDAI0B02960)
TPF: TPHA_0H00180(TPHA0H00180)
TBL: TBLA_0A08760(TBLA0A08760)
TDL: TDEL_0B04430(TDEL0B04430)
KAF: KAFR_0C02780(KAFR0C02780)
KNG: KNAG_0G01490(KNAG0G01490)
LEL: PVL30_002674(PIM1)
CAL: CAALFM_CR04340WA(PIM1)
SLB: AWJ20_2088(PIM1)
NCR: NCU05261
NTE: NEUTE1DRAFT61761(NEUTE1DRAFT_61761)
PBEL: QC761_304170(PIM1)
PPSD: QC762_304170(PIM1)
PPSP: QC763_304170(PIM1)
PPSA: QC764_304170(PIM1)
MGR: MGG_11141
PGRI: PgNI_03704
SSCK: SPSK_02974
FPOA: FPOAC1_009167(PIM1)
FMU: J7337_004633(PIM1)
MAW: MAC_01341
MAJ: MAA_06367
CMT: CCM_02548
PTKZ: JDV02_000252(PIM1)
BFU: BCIN_07g06160(Bcpim1)
MBE: MBM_01738
ALUC: AKAW2_21299S(PIM1)
ACHE: ACHE_40930A(PIM1)
APUU: APUU_51126A(PIM1)
ABE: ARB_01104
TVE: TRV_04140
PTE: PTT_07258
SPO: SPAC22F3.06c(lon1)
SOM: SOMG_01542(lon1)
CNE: CND03865
CNB: CNBD2440
CDEU: CNBG_0834
TASA: A1Q1_01255
CCAC: CcaHIS019_0604850(PIM1)
ABP: AGABI1DRAFT75578(AGABI1DRAFT_75578)
ABV: AGABI2DRAFT202009(AGABI2DRAFT_202009)
MORE: E1B28_010740(PIM1)
MGL: MGL_4002
MRT: MRET_4237
TAN: TA12745
TPV: TP02_0361
AHEL: Q31a_24800(lon_1)
 » show all
Reference
  Authors
Bota DA, Davies KJ
  Title
Lon protease preferentially degrades oxidized mitochondrial aconitase by an ATP-stimulated mechanism.
  Journal
Nat Cell Biol 4:674-80 (2002)
DOI:10.1038/ncb836
Reference
  Authors
Lu B, Yadav S, Shah PG, Liu T, Tian B, Pukszta S, Villaluna N, Kutejova E, Newlon CS, Santos JH, Suzuki CK
  Title
Roles for the human ATP-dependent Lon protease in mitochondrial DNA maintenance.
  Journal
J Biol Chem 282:17363-74 (2007)
DOI:10.1074/jbc.M611540200
  Sequence
[hsa:9361]
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