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SPG7 SPG7 AFG3L2 AFG3L2 CLPP CLPP USP2 USP2 CLPX CLPX UBC UBC LONP1 LONP1 ACO2 ACO2 IDH3A IDH3A PSMD5 PSMD5 PSMC3 PSMC3
"LONP1" - lon peptidase 1, mitochondrial in Homo sapiens
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protein of unknown 3D structure
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Known Interactions
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Predicted Interactions
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protein homology
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LONP1lon peptidase 1, mitochondrial; 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 mitocho [...] (959 aa)    
Predicted Functional Partners:
CLPP
ClpP caseinolytic peptidase, ATP-dependent, proteolytic subunit homolog (E. coli); Clp cleaves peptides in various proteins in a process that requires ATP hydrolysis. Clp may be responsible for a fairly general and central housekeeping function rather than for the degradation of specific substrates (277 aa)
score_image     score_image score_image   score_image   0.806
AFG3L2
AFG3 ATPase family member 3-like 2 (S. cerevisiae); ATP-dependent protease which is essential for axonal development (By similarity) (797 aa)
      score_image score_image   score_image   0.784
ACO2
aconitase 2, mitochondrial; Catalyzes the isomerization of citrate to isocitrate via cis-aconitate (By similarity) (780 aa)
            score_image   0.767
PSMD5
proteasome (prosome, macropain) 26S subunit, non-ATPase, 5; Acts as a chaperone during the assembly of the 26S proteasome, specifically of the base subcomplex of the PA700/19S regulatory complex (RC). In the initial step of the base subcomplex assembly is part of an intermediate PSMD5-PSMC2-PSMC1-PSMD2 module which probably assembles with a PSMD10-PSMC4-PSMC5-PAAF1 module followed by dissociation of PSMD5 (504 aa)
            score_image   0.757
USP2
ubiquitin specific peptidase 2; Hydrolase that deubiquitinates polyubiquitinated target proteins such as MDM2, MDM4 and CCND1. Isoform 1 and isoform 4 possess both ubiquitin-specific peptidase and isopeptidase activities. Deubiquitinates MDM2 without reversing MDM2-mediated p53/TP53 ubiquitination and thus indirectly promotes p53/TP53 degradation and limits p53 activity. Has no deubiquitinase activity against p53/TP53. Prevents MDM2-mediated degradation of MDM4. Plays a role in the G1/S cell-cycle progression in normal and cancer cells. Plays a role in the regulation of myogenic differ [...] (605 aa)
        score_image       0.740
CLPX
ClpX caseinolytic peptidase X homolog (E. coli); ATP-dependent specificity component of the Clp protease. It directs the protease to specific substrates. Can perform chaperone functions in the absence of clpP (By similarity) (633 aa)
score_image     score_image     score_image   0.737
SPG7
spastic paraplegia 7 (pure and complicated autosomal recessive); Putative ATP-dependent zinc metalloprotease (795 aa)
      score_image score_image   score_image   0.719
UBC
ubiquitin C (685 aa)
        score_image       0.696
IDH3A
isocitrate dehydrogenase 3 (NAD+) alpha (366 aa)
        score_image   score_image   0.695
PSMC3
proteasome (prosome, macropain) 26S subunit, ATPase, 3; The 26S protease is involved in the ATP-dependent degradation of ubiquitinated proteins. The regulatory (or ATPase) complex confers ATP dependency and substrate specificity to the 26S complex (By similarity). In case of HIV-1 infection, suppresses Tat-mediated transactivation (439 aa)
        score_image   score_image   0.673
Your Current Organism:
Homo sapiens
NCBI taxonomy Id: 9606
Other names: H. sapiens, Homo, Homo sapiens, human, man
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