Lon protease homolog, mitochondrial

<p>This section provides any useful information about the protein, mostly biological knowledge.<p><a href='/help/function_section' target='_top'>More...</a></p>Functionⁱ

<p>This subsection of the <a href="http://www.uniprot.org/help/function%5Fsection">Function</a> section describes the catalytic activity of an enzyme, i.e. a chemical reaction that the enzyme catalyzes.<p><a href='/help/catalytic_activity' target='_top'>More...</a></p>Catalytic activityⁱ

• Hydrolysis of proteins in presence of ATP.UniRule annotation

  Manual assertion according to rulesⁱ

  • HAMAP-Rule:MF_03120

  EC:3.4.21.53

Sites

Regions

<p>The <a href="http://www.geneontology.org/">Gene Ontology (GO)</a> project provides a set of hierarchical controlled vocabulary split into 3 categories:<p><a href='/help/gene_ontology' target='_top'>More...</a></p>GO - Molecular functionⁱ

• ADP binding Source: RGDInferred from sequence orthologyⁱ
  • "DNA and RNA binding by the mitochondrial lon protease is regulated by nucleotide and protein substrate."
    Liu T., Lu B., Lee I., Ondrovicova G., Kutejova E., Suzuki C.K.
    J. Biol. Chem. 279:13902-13910(2004) [PubMed] [Europe PMC] [Abstract]
• ATPase activity Source: RGDInferred from sequence orthologyⁱ
  • "The ATP-dependent Lon protease of Mus musculus is a DNA-binding protein that is functionally conserved between yeast and mammals."
    Lu B., Liu T., Crosby J.A., Thomas-Wohlever J., Lee I., Suzuki C.K.
    Gene 306:45-55(2003) [PubMed] [Europe PMC] [Abstract]
  • "DNA and RNA binding by the mitochondrial lon protease is regulated by nucleotide and protein substrate."
    Liu T., Lu B., Lee I., Ondrovicova G., Kutejova E., Suzuki C.K.
    J. Biol. Chem. 279:13902-13910(2004) [PubMed] [Europe PMC] [Abstract]
• ATP binding Source: RGDInferred from sequence orthologyⁱ
  • "DNA and RNA binding by the mitochondrial lon protease is regulated by nucleotide and protein substrate."
    Liu T., Lu B., Lee I., Ondrovicova G., Kutejova E., Suzuki C.K.
    J. Biol. Chem. 279:13902-13910(2004) [PubMed] [Europe PMC] [Abstract]
• ATP-dependent peptidase activity Source: RGD <p>Inferred from Direct Assay</p> <p>Used to indicate a direct assay for the function, process or component indicated by the GO term.</p> <p>More information in the <a href="http://geneontology.org/page/guide%2Dgo%2Devidence%2Dcodes#ida">GO evidence code guide</a></p> Inferred from direct assayⁱ
  • Ref.3

    "Changes in rat liver mitochondria with aging. Lon protease-like reactivity and N(epsilon)-carboxymethyllysine accumulation in the matrix."
    Bakala H., Delaval E., Hamelin M., Bismuth J., Borot-Laloi C., Corman B., Friguet B.
    Eur. J. Biochem. 270:2295-2302(2003) [PubMed] [Europe PMC] [Abstract]

    Cited for: FUNCTION, SUBCELLULAR LOCATION.
• DNA polymerase binding Source: RGDInferred from sequence orthologyⁱ
  • "DNA and RNA binding by the mitochondrial lon protease is regulated by nucleotide and protein substrate."
    Liu T., Lu B., Lee I., Ondrovicova G., Kutejova E., Suzuki C.K.
    J. Biol. Chem. 279:13902-13910(2004) [PubMed] [Europe PMC] [Abstract]
• G-quadruplex DNA binding Source: RGDInferred from sequence orthologyⁱ
  • "Thermodynamic characterization of specific interactions between the human Lon protease and G-quartet DNA."
    Chen S.H., Suzuki C.K., Wu S.H.
    Nucleic Acids Res. 36:1273-1287(2008) [PubMed] [Europe PMC] [Abstract]
• identical protein binding Source: RGDInferred from sequence orthologyⁱ
  • "DNA and RNA binding by the mitochondrial lon protease is regulated by nucleotide and protein substrate."
    Liu T., Lu B., Lee I., Ondrovicova G., Kutejova E., Suzuki C.K.
    J. Biol. Chem. 279:13902-13910(2004) [PubMed] [Europe PMC] [Abstract]
• mitochondrial promoter sequence-specific DNA binding Source: RGDInferred from sequence orthologyⁱ
  • "The ATP-dependent Lon protease of Mus musculus is a DNA-binding protein that is functionally conserved between yeast and mammals."
    Lu B., Liu T., Crosby J.A., Thomas-Wohlever J., Lee I., Suzuki C.K.
    Gene 306:45-55(2003) [PubMed] [Europe PMC] [Abstract]
  • "The human LON protease binds to mitochondrial promoters in a single-stranded, site-specific, strand-specific manner."
    Fu G.K., Markovitz D.M.
    Biochemistry 37:1905-1909(1998) [PubMed] [Europe PMC] [Abstract]
• sequence-specific DNA binding Source: RGDInferred from sequence orthologyⁱ
  • "DNA and RNA binding by the mitochondrial lon protease is regulated by nucleotide and protein substrate."
    Liu T., Lu B., Lee I., Ondrovicova G., Kutejova E., Suzuki C.K.
    J. Biol. Chem. 279:13902-13910(2004) [PubMed] [Europe PMC] [Abstract]
• serine-type endopeptidase activity Source: UniProtKB-UniRule
• single-stranded DNA binding Source: RGDInferred from sequence orthologyⁱ
  • "The ATP-dependent Lon protease of Mus musculus is a DNA-binding protein that is functionally conserved between yeast and mammals."
    Lu B., Liu T., Crosby J.A., Thomas-Wohlever J., Lee I., Suzuki C.K.
    Gene 306:45-55(2003) [PubMed] [Europe PMC] [Abstract]
• single-stranded RNA binding Source: RGDInferred from sequence orthologyⁱ
  • "DNA and RNA binding by the mitochondrial lon protease is regulated by nucleotide and protein substrate."
    Liu T., Lu B., Lee I., Ondrovicova G., Kutejova E., Suzuki C.K.
    J. Biol. Chem. 279:13902-13910(2004) [PubMed] [Europe PMC] [Abstract]

GO - Biological processⁱ

• aging Source: RGD <p>Inferred from Expression Pattern</p> <p>Covers cases where the annotation is inferred from the timing or location of expression of a gene.</p> <p>More information in the <a href="http://geneontology.org/page/guide%2Dgo%2Devidence%2Dcodes#iep">GO evidence code guide</a></p> Inferred from expression patternⁱ
  • Ref.4

    "Age-related impairment of mitochondrial matrix aconitase and ATP-stimulated protease in rat liver and heart."
    Delaval E., Perichon M., Friguet B.
    Eur. J. Biochem. 271:4559-4564(2004) [PubMed] [Europe PMC] [Abstract]

    Cited for: FUNCTION, SUBCELLULAR LOCATION.
• cellular protein-containing complex assembly Source: RGD <p>Inferred from Mutant Phenotype</p> <p>Describes annotations that are concluded from looking at variations or changes in a gene product such as mutations or abnormal levels and includes techniques such as knockouts, overexpression, anti-sense experiments and use of specific protein inhibitors.</p> <p>More information in the <a href="http://geneontology.org/page/guide%2Dgo%2Devidence%2Dcodes#imp">GO evidence code guide</a></p> Inferred from mutant phenotypeⁱ
  • Ref.1

    "Transmission of cell stress from endoplasmic reticulum to mitochondria: enhanced expression of Lon protease."
    Hori O., Ichinoda F., Tamatani T., Yamaguchi A., Sato N., Ozawa K., Kitao Y., Miyazaki M., Harding H.P., Ron D., Tohyama M., Stern D.M., Ogawa S.
    J. Cell Biol. 157:1151-1160(2002) [PubMed] [Europe PMC] [Abstract]

    Cited for: NUCLEOTIDE SEQUENCE [MRNA], INDUCTION.
• cellular response to oxidative stress Source: RGDInferred from sequence orthologyⁱ
  • "Roles for the human ATP-dependent Lon protease in mitochondrial DNA maintenance."
    Lu B., Yadav S., Shah P.G., Liu T., Tian B., Pukszta S., Villaluna N., Kutejova E., Newlon C.S., Santos J.H., Suzuki C.K.
    J. Biol. Chem. 282:17363-17374(2007) [PubMed] [Europe PMC] [Abstract]
• chaperone-mediated protein complex assembly Source: GO_Central <p>Inferred from Biological aspect of Ancestor</p> <p>A type of phylogenetic evidence whereby an aspect of a descendent is inferred through the characterization of an aspect of a ancestral gene.</p> <p>More information in the <a href="http://geneontology.org/page/guide%2Dgo%2Devidence%2Dcodes#iba">GO evidence code guide</a></p> Inferred from biological aspect of ancestorⁱ
  • "Phylogenetic-based propagation of functional annotations within the Gene Ontology consortium."
    Gaudet P., Livstone M.S., Lewis S.E., Thomas P.D.
    Brief. Bioinformatics 12:449-462(2011) [PubMed] [Europe PMC] [Abstract]
• mitochondrion organization Source: RGDInferred from expression patternⁱ
  • "Enhanced mitochondrial biogenesis is associated with increased expression of the mitochondrial ATP-dependent Lon protease."
    Luciakova K., Sokolikova B., Chloupkova M., Nelson B.D.
    FEBS Lett. 444:186-188(1999) [PubMed] [Europe PMC] [Abstract]
• oxidation-dependent protein catabolic process Source: RGDInferred from sequence orthologyⁱ
  • "Lon protease preferentially degrades oxidized mitochondrial aconitase by an ATP-stimulated mechanism."
    Bota D.A., Davies K.J.
    Nat. Cell Biol. 4:674-680(2002) [PubMed] [Europe PMC] [Abstract]
• protein quality control for misfolded or incompletely synthesized proteins Source: GO_CentralInferred from biological aspect of ancestorⁱ
  • "Phylogenetic-based propagation of functional annotations within the Gene Ontology consortium."
    Gaudet P., Livstone M.S., Lewis S.E., Thomas P.D.
    Brief. Bioinformatics 12:449-462(2011) [PubMed] [Europe PMC] [Abstract]
• proteolysis Source: RGDInferred from direct assayⁱ
  • Ref.3

    "Changes in rat liver mitochondria with aging. Lon protease-like reactivity and N(epsilon)-carboxymethyllysine accumulation in the matrix."
    Bakala H., Delaval E., Hamelin M., Bismuth J., Borot-Laloi C., Corman B., Friguet B.
    Eur. J. Biochem. 270:2295-2302(2003) [PubMed] [Europe PMC] [Abstract]

    Cited for: FUNCTION, SUBCELLULAR LOCATION.
• proteolysis involved in cellular protein catabolic process Source: RGDInferred from sequence orthologyⁱ
  • "Roles for the human ATP-dependent Lon protease in mitochondrial DNA maintenance."
    Lu B., Yadav S., Shah P.G., Liu T., Tian B., Pukszta S., Villaluna N., Kutejova E., Newlon C.S., Santos J.H., Suzuki C.K.
    J. Biol. Chem. 282:17363-17374(2007) [PubMed] [Europe PMC] [Abstract]
  • "A human mitochondrial ATP-dependent protease that is highly homologous to bacterial Lon protease."
    Wang N., Gottesman S., Willingham M.C., Gottesman M.M., Maurizi M.R.
    Proc. Natl. Acad. Sci. U.S.A. 90:11247-11251(1993) [PubMed] [Europe PMC] [Abstract]
• response to aluminum ion Source: RGDInferred from expression patternⁱ
  • "Susceptibility of mitochondrial superoxide dismutase to aluminium induced oxidative damage."
    Kumar V., Bal A., Gill K.D.
    Toxicology 255:117-123(2009) [PubMed] [Europe PMC] [Abstract]
• response to hormone Source: RGDInferred from expression patternⁱ
  • "Enhanced mitochondrial biogenesis is associated with increased expression of the mitochondrial ATP-dependent Lon protease."
    Luciakova K., Sokolikova B., Chloupkova M., Nelson B.D.
    FEBS Lett. 444:186-188(1999) [PubMed] [Europe PMC] [Abstract]
• response to hypoxia Source: RGDInferred from expression patternⁱ
  • Ref.1

    "Transmission of cell stress from endoplasmic reticulum to mitochondria: enhanced expression of Lon protease."
    Hori O., Ichinoda F., Tamatani T., Yamaguchi A., Sato N., Ozawa K., Kitao Y., Miyazaki M., Harding H.P., Ron D., Tohyama M., Stern D.M., Ogawa S.
    J. Cell Biol. 157:1151-1160(2002) [PubMed] [Europe PMC] [Abstract]

    Cited for: NUCLEOTIDE SEQUENCE [MRNA], INDUCTION.

<p>UniProtKB Keywords constitute a <a href="http://www.uniprot.org/keywords">controlled vocabulary</a> with a hierarchical structure. Keywords summarise the content of a UniProtKB entry and facilitate the search for proteins of interest.<p><a href='/help/keywords' target='_top'>More...</a></p>Keywordsⁱ

Protein family/group databases

<p>This section provides information about the protein and gene name(s) and synonym(s) and about the organism that is the source of the protein sequence.<p><a href='/help/names_and_taxonomy_section' target='_top'>More...</a></p>Names & Taxonomyⁱ

Organism-specific databases

<p>This section provides information on the location and the topology of the mature protein in the cell.<p><a href='/help/subcellular_location_section' target='_top'>More...</a></p>Subcellular locationⁱ

Keywords - Cellular componentⁱ

<p>This section describes post-translational modifications (PTMs) and/or processing events.<p><a href='/help/ptm_processing_section' target='_top'>More...</a></p>PTM / Processingⁱ

Molecule processing

Proteomic databases

PTM databases

<p>This section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms.<p><a href='/help/expression_section' target='_top'>More...</a></p>Expressionⁱ

<p>This subsection of the 'Expression' section reports the experimentally proven effects of inducers and repressors (usually chemical compounds or environmental factors) on the level of protein (or mRNA) expression (up-regulation, down-regulation, constitutive expression).<p><a href='/help/induction' target='_top'>More...</a></p>Inductionⁱ

Gene expression databases

<p>This section provides information on the quaternary structure of a protein and on interaction(s) with other proteins or protein complexes.<p><a href='/help/interaction_section' target='_top'>More...</a></p>Interactionⁱ

<p>This subsection of the <a href="http://www.uniprot.org/help/interaction%5Fsection">'Interaction'</a> section provides information about the protein quaternary structure and interaction(s) with other proteins or protein complexes (with the exception of physiological receptor-ligand interactions which are annotated in the <a href="http://www.uniprot.org/help/function%5Fsection">'Function'</a> section).<p><a href='/help/subunit_structure' target='_top'>More...</a></p>Subunit structureⁱ

GO - Molecular functionⁱ

• DNA polymerase binding Source: RGDInferred from sequence orthologyⁱ
  • "DNA and RNA binding by the mitochondrial lon protease is regulated by nucleotide and protein substrate."
    Liu T., Lu B., Lee I., Ondrovicova G., Kutejova E., Suzuki C.K.
    J. Biol. Chem. 279:13902-13910(2004) [PubMed] [Europe PMC] [Abstract]
• identical protein binding Source: RGDInferred from sequence orthologyⁱ
  • "DNA and RNA binding by the mitochondrial lon protease is regulated by nucleotide and protein substrate."
    Liu T., Lu B., Lee I., Ondrovicova G., Kutejova E., Suzuki C.K.
    J. Biol. Chem. 279:13902-13910(2004) [PubMed] [Europe PMC] [Abstract]

Protein-protein interaction databases

<p>This section provides information on the tertiary and secondary structure of a protein.<p><a href='/help/structure_section' target='_top'>More...</a></p>Structureⁱ

3D structure databases

<p>This section provides information on sequence similarities with other proteins and the domain(s) present in a protein.<p><a href='/help/family_and_domains_section' target='_top'>More...</a></p>Family & Domainsⁱ

Domains and Repeats

<p>This subsection of the 'Family and domains' section provides information about the sequence similarity with other proteins.<p><a href='/help/sequence_similarities' target='_top'>More...</a></p>Sequence similaritiesⁱ

Keywords - Domainⁱ

Phylogenomic databases

Family and domain databases

<p>This section displays by default the canonical protein sequence and upon request all isoforms described in the entry. It also includes information pertinent to the sequence(s), including <a href="http://www.uniprot.org/help/sequence%5Flength">length</a> and <a href="http://www.uniprot.org/help/sequences">molecular weight</a>. The information is filed in different subsections. The current subsections and their content are listed below:<p><a href='/help/sequences_section' target='_top'>More...</a></p>Sequenceⁱ

<p>This subsection of the <a href="http://www.uniprot.org/help/sequences%5Fsection">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical%5Fand%5Fisoforms">canonical sequence</a> displayed by default in the entry is complete or not.<p><a href='/help/sequence_status' target='_top'>More...</a></p>Sequence statusⁱ: Complete.

<p>This subsection of the <a href="http://www.uniprot.org/help/sequences%5Fsection">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical%5Fand%5Fisoforms">canonical sequence</a> displayed by default in the entry is in its mature form or if it represents the precursor.<p><a href='/help/sequence_processing' target='_top'>More...</a></p>Sequence processingⁱ: The displayed sequence is further processed into a mature form.

        10         20         30         40         50
MAASTGYVRL WAAARCWVLR RPLLAVTGGR VPSASGSWLR RGCRVCDTST 
        60         70         80         90        100
PWGGRVPMGG GQWRGLWDAG SRGGSDETSE GGVEDGATAS SGEGPVVTAL 
       110        120        130        140        150
APMTVPDVFP HLPLIAISRN PVFPRFIKIV EVKNKKLVEL LRRKVRLAQP 
       160        170        180        190        200
YVGVFLKRDD NNESDVVESL DEIYHTGTFA QIHEMQDLGD KLRMIVTGHR 
       210        220        230        240        250
RIHISRQLEV EPEGLEPEAE NKQKSRRKLK RGKKEVGDEL GAKPQLEMVT 
       260        270        280        290        300
EATSDTSKEV LMVEVENVAH EDFQVTEEVK ALTAEIVKTI RDIIALNPLY 
       310        320        330        340        350
RESVLQMMQA GQRVVDNPIY LSDMGAALTG AESHELQDVL EETNILKRLY 
       360        370        380        390        400
KALSLLKKEF ELSKLQQRLG REVEEKIKQT HRKYLLQEQL KIIKKELGLE 
       410        420        430        440        450
KDDKDAIEEK FRERLKELVV PKHVMDVVDE ELSKLALLDN HSSEFNVTRN 
       460        470        480        490        500
YLDWLTSIPW GRQSDENLDL ARAQSVLEED HYGMEDVKKR VLEFIAVSQL 
       510        520        530        540        550
RGSTQGKILC FHGPPGVGKT SIARSIARAL GREYFRFSVG GMTDVAEIKG 
       560        570        580        590        600
HRRTYVGAMP GKIIQCLKKT KTENPLVLID EVDKIGRGYQ GDPSSALLEL 
       610        620        630        640        650
LDPEQNANFL DHYLDVPVDL SKVLFICTAN VTDTIPEPLR DRMEMINVSG 
       660        670        680        690        700
YVAQEKLAIA ERYLVPQART LCGLDESKAQ LSATVLTLLI KQYCRESGVR 
       710        720        730        740        750
NLQKQVEKVL RKAAYKIVSG EAQTVHVTPE NLQDFVGKPV FTVERMYDVT 
       760        770        780        790        800
PPGVVMGLAW TAMGGSTLFV ETSLRRPQPS GSKEDKDGSL EVTGQLGDVM 
       810        820        830        840        850
KESARIAYTF ARAFLMEQDP ENDFLVTSHI HLHVPEGATP KDGPSAGCTI 
       860        870        880        890        900
VTALLSLALG QPVLQNLAMT GEVSLTGKVL PVGGIKEKTI AAKRAGVTCI 
       910        920        930        940        950
ILPAENRKDF SDLAPFITEG LEVHFVEHYR DIFRIAFPLR EHQEALAVER 

Sequence databases

Genome annotation databases

<p>This section provides links to proteins that are similar to the protein sequence(s) described in this entry at different levels of sequence identity thresholds (100%, 90% and 50%) based on their membership in UniProt Reference Clusters (<a href="http://www.uniprot.org/help/uniref">UniRef</a>).<p><a href='/help/similar_proteins_section' target='_top'>More...</a></p>Similar proteinsⁱ

• 50% Identity

<p>This section is used to point to information related to entries and found in data collections other than UniProtKB.<p><a href='/help/cross_references_section' target='_top'>More...</a></p>Cross-referencesⁱ

Sequence databases

3D structure databases

Protein-protein interaction databases

Protein family/group databases

PTM databases

Proteomic databases

Genome annotation databases

Organism-specific databases

Phylogenomic databases

Miscellaneous databases

Gene expression databases

Family and domain databases

<p>This section provides general information on the entry.<p><a href='/help/entry_information_section' target='_top'>More...</a></p>Entry informationⁱ

<p>This section contains any relevant information that doesn't fit in any other defined sections<p><a href='/help/miscellaneous_section' target='_top'>More...</a></p>Miscellaneousⁱ

Keywords - Technical termⁱ

Documents

1. Peptidase families
   Classification of peptidase families and list of entries
2. SIMILARITY comments
   Index of protein domains and families