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LONP1 Gene

protein-coding   GIFtS: 58
GCID: GC19M005691

Lon Peptidase 1, Mitochondrial

(Previous names: protease, serine, 15)
(Previous symbol: PRSS15)
  See LONP1-related diseases
at  

(According to 1HGNC, 2Entrez Gene,
3UniProtKB/Swiss-Prot, 4UniProtKB/TrEMBL, 5OMIM, 6GeneLoc, 7Ensembl, 8DME, 9miRBase, 10fRNAdb, 12H-InvDB, 13NCBI, 14NONCODE, and/or 15RNAdb)
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Aliases
Lon Peptidase 1, Mitochondrial1 2     hLON2
PRSS151 2 3 5     HLON ATP-Dependent Protease2
Mitochondrial ATP-Dependent Protease Lon2 3     Lon Protease Homolog, Mitochondrial2
Serine Protease 152 3     Mitochondrial Lon Protease-Like Protein2
LONP2 3     EC 3.4.21.-3
LON2 5     LONHs3
Protease, Serine, 151     Lon Protease-Like Protein3
LonHS2     EC 3.4.218
PIM12     EC 3.4.21.538

External Ids:    HGNC: 94791   Entrez Gene: 93612   Ensembl: ENSG000001963657   OMIM: 6054905   UniProtKB: P367763   

Export aliases for LONP1 gene to outside databases

Previous GC identifers: GC19M005643 GC19M005452


(According to Entrez Gene, GeneCards, Tocris Bioscience, Wikipedia's Gene Wiki, PharmGKB,
UniProtKB/Swiss-Prot, and/or UniProtKB/TrEMBL)
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Entrez Gene summary for LONP1 Gene:
This gene encodes a mitochondrial matrix protein that belongs to the Lon family of ATP-dependent proteases. This
protein mediates the selective degradation of misfolded, unassembled or oxidatively damaged polypeptides in the
mitochondrial matrix. It may also have a chaperone function in the assembly of inner membrane protein complexes,
and participate in the regulation of mitochondrial gene expression and maintenance of the integrity of the
mitochondrial genome. Decreased expression of this gene has been noted in a patient with hereditary spastic
paraplegia (PMID:18378094). Alternatively spliced transcript variants have been found for this gene. (provided by
RefSeq, Feb 2013)

GeneCards Summary for LONP1 Gene:
LONP1 (lon peptidase 1, mitochondrial) is a protein-coding gene. Diseases associated with LONP1 include glossopharyngeal neuralgia, and merrf syndrome. GO annotations related to this gene include single-stranded DNA binding and sequence-specific DNA binding. An important paralog of this gene is LONP2.

UniProtKB/Swiss-Prot: LONM_HUMAN, P36776
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.
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. Endogenous
substrates include mitochondrial steroidogenic acute regulatory (StAR) protein

summary for LONP1 Gene:
Pim kinases are constitutively active serine/threonine kinases that promote growth factor-independent
proliferation by phosphorylating, and thus inhibiting, a range of cellular proteins. Both Pim-1 and Pim-2
kinase are serine/threonine kinases that are involved in the control of cell growth, differentiation and
apoptosis.

Gene Wiki entry for LONP1 Gene


(According to GeneLoc and/or HGNC, and/or
Entrez Gene (NCBI build 37),
and/or miRBase,
Genomic Views according to UCSC (hg19) and Ensembl (release 75), Regulatory elements and Epigenetics data according to QIAGEN, and/or SwitchGear Genomics)
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RefSeq DNA sequence at NCBI GenBank:
NC_000019.9  NC_018930.2  NT_011295.12  
Regulatory elements:
   Regulatory transcription factor binding sites in the LONP1 gene promoter:
         Max1   ER-alpha   NF-1   NF-1/L   GATA-2   HEN1   Ik-2   POU2F1   POU2F1a   c-Myb   
         Other transcription factors

SwitchGear Promoter luciferase reporter plasmidLONP1 promoter sequence
   Search Chromatin IP Primers for LONP1

Epigenetics:
DNA Methylation CpG Assay Predesigned for Pyrosequencing in human, mouse, rat LONP1


Genomic Location:
Genomic View: UCSC Golden Path with GeneCards custom track

Entrez Gene cytogenetic band: 19p13.2   Ensembl cytogenetic band:  19p13.3   HGNC cytogenetic band: 19p13.2

LONP1 Gene in genomic location: bands according to Ensembl, locations according to (and/or Entrez Gene and/or Ensembl if different)
LONP1 gene location

GeneLoc information about chromosome 19         GeneLoc Exon Structure

GeneLoc location for GC19M005691:  view genomic region     (about GC identifiers)

Start:
5,691,845 bp from pter      End:
5,720,583 bp from pter
Size:
28,739 bases      Orientation:
minus strand

(According to 1UniProtKB, HORDE, 2neXtProt, Ensembl, and/or Reactome, Modification sites according to PhosphoSitePlus, Specific Peptides from DME, RefSeq according to NCBI, PDB rendering according to OCA and/or Proteopedia, Recombinant Proteins from EMD Millipore, R&D Systems, GenScript, Enzo Life Sciences, OriGene, Novus Biologicals, Sino Biological, ProSpec, Cloud-Clone Corp., and/or eBioscience,
Biochemical Assays by EMD Millipore, R&D Systems, OriGene, GenScript, Cell Signaling Technology, Enzo Life Sciences, Cloud-Clone Corp., and/or eBioscience, Antibodies by EMD Millipore, R&D Systems, Cell Signaling Technology, OriGene, Novus Biologicals, Thermo Fisher Scientific, LSBio, Abcam, Cloud-Clone Corp, and/or others.)
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UniProtKB/Swiss-Prot: LONM_HUMAN, P36776 (See protein sequence)
Recommended Name: Lon protease homolog, mitochondrial precursor  
Size: 959 amino acids; 106489 Da
Subunit: Homohexamer or homoheptamer. Organized in a ring with a central cavity. DNA and RNA binding is stimulated
by substrate and inhibited by ATP binding. Interacts with PEO1 and mitochondrial DNA polymerase subunit POLG
Sequence caution: Sequence=CAA52291.1; Type=Erroneous initiation;
1 PDB 3D structure from and Proteopedia for LONP1:
2X36 (3D)    
Secondary accessions: D6W635 E5KMH8 P36777 Q9UQ95

Explore the universe of human proteins at neXtProt for LONP1: NX_P36776

Explore proteomics data for LONP1 at MOPED

Post-translational modifications: 

  • Modification sites at neXtProt
  • Modification sites at PhosphoSitePlus
  • Selected DME Specific Peptides for LONP1 (P36776) (see all 21)
     KDGPSAG  DLSKVLF  LPVGGIK  PLRDRME 


    See LONP1 Protein Expression from SPIRE MOPED, PaxDB, and MaxQB

    REFSEQ proteins (3 alternative transcripts): 
    NP_001263408.1  NP_001263409.1  NP_004784.2  

    ENSEMBL proteins: 
     ENSP00000353826   ENSP00000467808   ENSP00000465585   ENSP00000468541   ENSP00000441523  
     ENSP00000465139   ENSP00000468379   ENSP00000468083   ENSP00000468114   ENSP00000468385  

    LONP1 Human Recombinant Protein Products:

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    Novus Biologicals LONP1 Lysates
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    Cloud-Clone Corp. Proteins for LONP1

     
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    LSBio Antibodies in human, mouse, rat for LONP1

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    Cloud-Clone Corp. ELISAs for LONP1
    Cloud-Clone Corp. CLIAs for LONP1
    Search eBioscience for ELISAs for LONP1 


    (According to HGNC, IUPHAR, InterPro, ProtoNet, UniProtKB, and/or BLOCKS, Sets of similar genes according to GeneDecks)
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    HGNC Gene Families:
    PRSS: Serine peptidases / Serine peptidases
    AATP: ATPases / AAA-type

    Selected InterPro protein domains (see all 12):
     IPR027503 Lonm_euk
     IPR003959 ATPase_AAA_core
     IPR015947 PUA-like_domain
     IPR004815 Lon_bac/euk-typ
     IPR008268 Peptidase_S16_AS

    Graphical View of Domain Structure for InterPro Entry P36776

    ProtoNet protein and cluster: P36776

    1 Blocks protein domain: IPB001984 Peptidase family S16

    UniProtKB/Swiss-Prot: LONM_HUMAN, P36776
    Similarity: Belongs to the peptidase S16 family
    Similarity: Contains 1 Lon domain


    Find genes that share domains with LONP1           About GenesLikeMe


    (According to 1UniProtKB, Genatlas, LifeMap Discovery™, IUBMB, and/or 2DME, Human phenotypes from GenomeRNAi, Animal models from MGI Mar 06 2013, inGenious Targeting Laboratory, genOway,
    transcription factor targeting from QIAGEN and/or HOMER, miRNA Gene Targets from miRTarBase, shRNA from OriGene, siRNAs from OriGene, QIAGEN, microRNA from QIAGEN, SwitchGear Genomics, Gene Editing from DNA2.0, Clones from OriGene, GenScript, Sino Biological, DNA2.0, Vector BioLabs and/or Addgene, Cell Lines from GenScript, ESI BIO, In Situ Hybridization Assays from Advanced Cell Diagnostics, Flow cytometry from eBioscience, Ontologies according to Gene Ontology Consortium 01 Apr 2014 via Entrez Gene.)
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    Molecular Function:

         UniProtKB/Swiss-Prot Summary: LONM_HUMAN, P36776
    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.
    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. Endogenous
    substrates include mitochondrial steroidogenic acute regulatory (StAR) protein

         Enzyme Numbers (IUBMB): EC 3.4.212 EC 3.4.21.532 EC 3.4.21.-1

         Gene Ontology (GO): Selected molecular function terms (see all 18):    About this table

    GO IDQualified GO termEvidencePubMed IDs
    GO:0000166nucleotide binding ----
    GO:0003697single-stranded DNA binding IBA--
    GO:0003727single-stranded RNA binding IDA14739292
    GO:0004176ATP-dependent peptidase activity IDA17420247
    GO:0004252serine-type endopeptidase activity IBA--
         
    Find genes that share ontologies with LONP1           About GenesLikeMe


    Phenotypes:
         8 GenomeRNAi human phenotypes for LONP1:
     Decreased Salmonella enterica   Decreased Salmonella enterica   Decreased Salmonella enterica   Decreased Salmonella enterica  
     Decreased Salmonella enterica   Decreased Salmonella-containin  Decreased influenza A H1N1 (A/  Decreased influenza A/WSN/33 r 

    Animal Models:
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       genOway customized Knockin model: humanization, point mutation, expression monitoring, etc. for LONP1

    miRNA
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    miRTarBase miRNAs that target LONP1:
    hsa-mir-16-5p (MIRT051178), hsa-mir-20a-3p (MIRT038977), hsa-mir-26b-5p (MIRT029135)

    Block miRNA regulation of human, mouse, rat LONP1 using miScript Target Protectors
    Search for qRT-PCR Assays for microRNAs that regulate LONP1
    SwitchGear 3'UTR luciferase reporter plasmidLONP1 3' UTR sequence
    Inhib. RNA
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    Predesigned siRNA for gene silencing in human, mouse, rat LONP1

    Gene Editing
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    GenScript: all cDNA clones in your preferred vector: LONP1 (NM_004793)
    Browse Sino Biological Human cDNA Clones
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    Advanced Cell Diagnostics RNAscope RNA in situ hybridization assays for LONP1


    (According to UniProtKB, COMPARTMENTS Subcellular localization database, Ontologies according to Gene Ontology Consortium 01 Apr 2014 via Entrez Gene.)
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    Subcellular locations from UniProtKB/Swiss-Prot
    LONM_HUMAN, P36776: Mitochondrion matrix
    Subcellular locations from COMPARTMENTS: 

    CompartmentConfidence
    mitochondrion5
    cytosol3
    peroxisome2
    nucleus1

    Gene Ontology (GO): 4 cellular component terms:    About this table

    GO IDQualified GO termEvidencePubMed IDs
    GO:0005737cytoplasm IDA--
    GO:0005739mitochondrion IDA--
    GO:0005759mitochondrial matrix IMP12198491
    GO:0042645mitochondrial nucleoid IDA18063578

    Find genes that share ontologies with LONP1           About GenesLikeMe


    (SuperPaths according to PathCards, Pathways according to R&D Systems, Cell Signaling Technology, KEGG, PharmGKB, BioSystems, Sino Biological, Reactome, Tocris Bioscience, GeneGo (Thomson Reuters), QIAGEN, and/or UniProtKB, Sets of similar genes according to GeneDecks, Interaction Networks according to QIAGEN, and/or STRING, Interactions according to 1UniProtKB, 2MINT, 3I2D, and/or 4STRING, with links to IntAct and Ensembl, Ontologies according to Gene Ontology Consortium 01 Apr 2014 via Entrez Gene).
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    3 Downloadable PowerPoint Slides of GeneGlobe Pathway Central Maps for LONP1
        STAT3 Pathway
    IL-10 Pathway
    IL-6 Pathway


        Custom Pathway & Disease-focused RT2 Profiler PCR Arrays for LONP1
    Interactions:

        Search GeneGlobe Interaction Network for LONP1

    STRING Interaction Network Preview (showing 5 interactants - click image to see 25)

    Selected Interacting proteins for LONP1 (P367762, 3 ENSP000003538264) via UniProtKB, MINT, STRING, and/or I2D (see all 361)
    InteractantInteraction Details
    GeneCardExternal ID(s)
    AFG3L2Q9Y4W62, 3, ENSP000002691434MINT-8079030 I2D: score=1 STRING: ENSP00000269143
    CLPXO760312, 3MINT-8079030 I2D: score=1 
    DAXXQ9UER73I2D: score=2 
    ENSG00000206206Q9UER73I2D: score=2 
    ENSG00000206279Q9UER73I2D: score=2 
    About this table

    Gene Ontology (GO): Selected biological process terms (see all 20):    About this table

    GO IDQualified GO termEvidencePubMed IDs
    GO:0000002mitochondrial genome maintenance NAS17420247
    GO:0001666response to hypoxia IEP17418790
    GO:0006200ATP catabolic process ----
    GO:0006281DNA repair ----
    GO:0006310DNA recombination ----

    Find genes that share ontologies with LONP1           About GenesLikeMe



    (Chemical Compounds according to UniProtKB, Enzo Life Sciences, EMD Millipore, Tocris Bioscience, ApexBio, HMDB, BitterDB, and/or Novoseek, Ligands according to IUPHAR, and Drugs according to DrugBank, Enzo Life Sciences, and/or PharmGKB)
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    Browse Small Molecules at EMD Millipore
       Browse drugs & compounds from Enzo Life Sciences
      Browse compounds at ApexBio 

    Compounds for LONP1 available from Tocris Bioscience    About this table
    CompoundAction CAS #
    PIM-1 Inhibitor 2Pim-1 kinase inhibitor[477845-12-8]
    TCS PIM-1 1 Selective, ATP-competitive Pim-1 kinase inhibitor [491871-58-0]
    R8-T198wtPim-1 kinase inhibitor--
    TCS PIM-1 4a Selective, ATP-competitive Pim kinase inhibitor [438190-29-5]

    3 Novoseek inferred chemical compound relationships for LONP1 gene    About this table
    Compound   -log (P-Val)   Hits   PubMed IDs for Articles with Shared Sentences (# sentences)
    serine 46.1 8 17497890 (2), 12698553 (2), 8119403 (1), 16819825 (1) (see all 6)
    atp 28.6 15 17497890 (3), 16819825 (3), 11262158 (2), 12657466 (1) (see all 8)
    oxygen 0 1 12198491 (1)



    Find genes that share compounds with LONP1           About GenesLikeMe



    (Secondary structures according to fRNAdb,
    GenBank/EMBL/DDBJ Accessions according to
    Unigene (Build 236 Homo sapiens; Apr 25 2013) or GenBank,
    RefSeq according to Entrez Gene,
    DOTS (version 10), and/or AceView, transcript ids from Ensembl with links to UCSC,
    exon structure from GeneLoc, alternative splicing isoforms according to ASD and/or ECgene,
    siRNAs from OriGene, QIAGEN, shRNA from OriGene, microRNA from QIAGEN, SwitchGear Genomics,
    Tagged/untagged cDNA clones from OriGene, GenScript, DNA2.0, Vector BioLabs, and/or Addgene, Primers from OriGene, and/or QIAGEN, Flow cytometry from eBioscience )
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    REFSEQ mRNAs for LONP1 gene (3 alternative transcripts): 
    NM_001276479.1  NM_001276480.1  NM_004793.3  

    Unigene Cluster for LONP1:

    Lon peptidase 1, mitochondrial
    Hs.350265  [show with all ESTs]
    Unigene Representative Sequence: NM_004793
    15 Ensembl transcripts including schematic representations, and UCSC links where relevant:
    ENST00000587552 ENST00000360614(uc002mcx.3 uc002mcy.3 uc010duh.3 uc010dui.3)
    ENST00000590558 ENST00000585374 ENST00000593119 ENST00000540670(uc002mcz.3)
    ENST00000590729 ENST00000589473 ENST00000590206 ENST00000588589 ENST00000587365
    ENST00000591321 ENST00000586617 ENST00000590728 ENST00000590511
    miRNA
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    Predesigned siRNA for gene silencing in human, mouse, rat LONP1
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      QuantiTect SYBR Green Assays in human, mouse, rat LONP1
      QuantiFast Probe-based Assays in human, mouse, rat LONP1

    Additional mRNA sequence: 

    AK056366.1 AK096433.1 AK096626.1 AK127867.1 AK298531.1 AK309720.1 BC000235.1 BC109218.1 
    BC109219.1 BC139726.1 NR_076392.1 U02389.1 

    Selected DOTS entries (see all 31):

    DT.453331  DT.100795852  DT.91646447  DT.95273443  DT.121494729  DT.100795851  DT.87017359  DT.100769574 
    DT.121494686  DT.95096720  DT.97770121  DT.100039305  DT.121494823  DT.100665362  DT.92407390  DT.95123988 
    DT.91834634  DT.91984921  DT.100795860  DT.121494819  DT.121494826  DT.100795859  DT.121494793  DT.75127239 

    Selected AceView cDNA sequences (see all 614):

    BM746164 BG027931 BU625223 AI917405 BM765209 T29819 AK127867 BM980493 
    BQ059488 BC000235 CR603800 BQ882407 BQ073278 BG761974 CA313685 BU169028 
    CA435958 BM825784 BU737502 BU680995 BE747273 BM804803 BQ001542 BM857003 

    GeneLoc Exon Structure

    Selected Alternative Splicing Database (ASD) splice patterns (SP) for LONP1 (see all 12)    About this scheme

    ExUns: 1a · 1b · 1c ^ 2a · 2b · 2c ^ 3a · 3b ^ 4 ^ 5 ^ 6 ^ 7a · 7b · 7c ^ 8a · 8b ^ 9a · 9b · 9c ^ 10 ^ 11 ^ 12a · 12b ^ 13 ^ 14a · 14b ^
    SP1:                                -                 -                                   -           -                                                         
    SP2:              -     -     -     -     -     -     -                                   -           -                                                         
    SP3:                                                  -                 -     -     -     -           -                                                         
    SP4:                                                  -                                   -     -     -                                                         
    SP5:                                                                                                                    -                                       

    ExUns: 15 ^ 16 ^ 17 ^ 18 ^ 19a · 19b ^ 20a · 20b ^ 21a · 21b · 21c · 21d
    SP1:                          -                 -     -                     
    SP2:                          -                 -     -                     
    SP3:                          -                 -     -                     
    SP4:                                                                        
    SP5:                                                                        


    ECgene alternative splicing isoforms for LONP1

    (RNA expression data according to H-InvDB, NONCODE, miRBase, and RNAdb, Expression images according to data from BioGPS, Illumina Human BodyMap, and CGAP SAGE, Sets of similar genes according to GeneDecks, in vivo and in vitro expression data from LifeMap Discovery™, Protein expression images according to data from SPIRE 1MOPED, 2PaxDb, and 3MaxQB, plus additional links to SOURCE, and/or BioGPS, and/or UniProtKB,
    PCR Arrays from QIAGEN, Primers from OriGene, and/or QIAGEN, In Situ Hybridization Assays from Advanced Cell Diagnostics)
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    LONP1 expression in normal human tissues (normalized intensities)
    See probesets specificity/sensitivity at GeneAnnot
    About this imageBioGPS <intensity>2/3
    CGAP TAG: ACTGGCGAAG
    LONP1 Expression
    About this image

    LONP1 Protein expression data from MOPED1, PaxDb2 and MaxQB3    About this image

    LONP1 Protein Expression

    SOURCE GeneReport for Unigene cluster: Hs.350265

    UniProtKB/Swiss-Prot: LONM_HUMAN, P36776
    Tissue specificity: Duodenum, heart, lung and liver, but not thymus

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    (Orthologs according to 1,2HomoloGene (2older version, for species not in 1newer version), 3euGenes, 4SGD , 5MGI Mar 06 2013, with possible further links to Flybase and/or WormBase, and/or 6Ensembl pan taxonomic compara , Gene Trees according to Ensembl and TreeFam)
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    This gene was present in the common ancestor of eukaryotes.

    Orthologs for LONP1 gene from Selected species (see all 25)    About this table
    Organism Taxonomic
    classification
    Gene Description Human
    Similarity
    Orthology
    Type
    Details
    mouse
    (Mus musculus)
    Mammalia Lonp11 , 5 lon peptidase 1, mitochondrial1, 5 82.49(n)1
    87.92(a)1
      17 (29.42 cM)5
    741421  NM_028782.21  NP_083058.21 
     566143015 
    chicken
    (Gallus gallus)
    Aves LONP11 lon peptidase 1, mitochondrial 78.08(n)
    88.47(a)
      768942  XM_001232111.3  XP_001232112.2 
    African clawed frog
    (Xenopus laevis)
    Amphibia Xl.240772 Xenopus laevis transcribed sequence with moderate similarity more 75.34(n)    BX842761.1 
    zebrafish
    (Danio rerio)
    Actinopterygii CD604580.12   -- 79.32(n)   325281  CD604580.1 
    fruit fly
    (Drosophila melanogaster)
    Insecta Lon1 Lon protease 63.79(n)
    67.71(a)
      40138  NM_140876.3  NP_649133.1 
    worm
    (Caenorhabditis elegans)
    Secernentea C34B2.61 C34B2.6 54.78(n)
    56.71(a)
      172966  NM_060395.5  NP_492796.1 
    baker's yeast
    (Saccharomyces cerevisiae)
    Saccharomycetes PIM1(YBL022C)4
    PIM11
    ATP-dependent Lon protease, involved in degradation more4
    PIM11
    50.82(n)1
    49.58(a)1
      2(181275-177874)4
    8522591, 4  NP_009531.11, 4 
    thale cress
    (Arabidopsis thaliana)
    eudicotyledons LON11 LON1 56.92(n)
    57.44(a)
      832744  NM_122568.3  NP_568490.3 
    rice
    (Oryza sativa)
    Liliopsida NM_185527.12   -- 71.42(n)    NM_185527.1 


    ENSEMBL Gene Tree for LONP1 (if available)
    TreeFam Gene Tree for LONP1 (if available)

    (Paralogs according to 1HomoloGene,
    2Ensembl, and 3SIMAP, Pseudogenes according to 4Pseudogene.org Build 68)
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    Paralogs for LONP1 gene
    LONP22  
    2 SIMAP similar genes for LONP1 using alignment to 17 protein entries:     LONM_HUMAN (see all proteins):
    LONP2    FLJ00373

    Find genes that share paralogs with LONP1           About GenesLikeMe



    (SNPs/Variants according to the 1NCBI SNP Database, 2Ensembl, 3PupaSUITE, 4UniProtKB, and DNA2.0, Linkage Disequilibrium by HapMap, Structural Variations(CNVs/InDels/Inversions) from the Database of Genomic Variants, Mutations from the Human Gene Mutation Database (HGMD), the Human Cytochrome P450 Allele Nomenclature Database, and the Locus Specific Mutation Databases (LSDB), Blood group antigen gene mutations by BGMUT, Resequencing Primers, Cancer Mutation PCR Arrays and Assays, and Copy Number PCR Arrays from QIAGEN)
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    Selected SNPs for LONP1 (see all 1049)    About this table                                 

    Genomic DataTranscription Related DataAllele Frequencies
    SNP IDValidClinical
    significance
    Chr 19 posSequence#AA
    Chg
    TypeMore#Allele
    freq
    PopTotal
    sample
    More
    ----------
    rs2011131211,2
    C--5615902(+) CACCAA/GGTTCG 4 -- ds50010--------
    rs115561101,2
    H--5615988(-) CCGTTT/CGTCCT 4 -- ds50016Minor allele frequency- C:0.00NS EA NA 406
    rs2003766771,2
    --5616060(+) GGGAGC/TCAGGG 4 -- ds50010--------
    rs2017483291,2
    C--5616068(+) GGGCCA/GGGCTG 4 -- ds50010--------
    rs455830311,2
    C,F--5616084(+) GGCCTC/TGTCCC 4 -- ds50013Minor allele frequency- T:0.05NA EU 713
    rs778532801,2
    C--5616089(+) CGTCCC/TTGGCA 4 -- ds50012Minor allele frequency- T:0.00WA EU 755
    rs2016218141,2
    C--5616194(+) CCTCCC/TCTGCC 4 -- ds50010--------
    rs2003650011,2
    --5616229(+) TGACAA/GAAGCC 4 -- ds50010--------
    rs1167391771,2
    F--5616257(+) CGTGGG/ATGGGT 4 -- ds50011Minor allele frequency- A:0.01WA 118
    rs1134958381,2
    C--5616357(+) CGTGGC/TCCGCC 4 -- ds50010--------

    HapMap Linkage Disequilibrium report for LONP1 (5691845 - 5720583 bp)

    Structural Variations
         Database of Genomic Variants (DGV) 7 variations for LONP1:    About this table    
    Variant IDTypeSubtypePubMed ID
    nsv521656CNV Loss19592680
    dgv3694n71CNV Loss21882294
    nsv470119CNV Loss18288195
    nsv910894CNV Loss21882294
    nsv516021CNV Loss19592680
    dgv3695n71CNV Loss21882294
    dgv3693n71CNV Loss21882294

    Site Specific Mutation Identification with PCR Assays
    SeqTarget long-range PCR primers for resequencing LONP1
    DNA2.0 Custom Variant and Variant Library Synthesis for LONP1

    (in which this Gene is Involved, According to MalaCards, OMIM, UniProtKB, the University of Copenhagen DISEASES database, Novoseek, Genatlas, GeneTests, GAD, HuGE Navigator, and/or TGDB.)
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    OMIM gene information: 605490    OMIM disorders: --

    2 diseases for LONP1:    
    About MalaCards
    glossopharyngeal neuralgia    merrf syndrome

    2 diseases from the University of Copenhagen DISEASES database for LONP1:
    Leishmaniasis     Glossopharyngeal neuralgia

    Find genes that share disorders with LONP1           About GenesLikeMe

    4 Novoseek inferred disease relationships for LONP1 gene    About this table

    Disease   -log (P-Val)   Hits   PubMed IDs for Articles with Shared Sentences (# sentences)
    shock 19.5 2 19439239 (1), 16428295 (1)
    breast cancer 0 2 20191845 (1), 17584658 (1)
    tumors 0 2 17584658 (1), 12372343 (1)
    cancer 0 1 16800730 (1)

    Genetic Association Database (GAD): LONP1
    Human Genome Epidemiology (HuGE) Navigator: LONP1 (1 document)

    Export disorders for LONP1 gene to outside databases

    (in PubMed. Associations of this gene to articles via 1Entrez Gene, 2UniProtKB/Swiss-Prot, 3HGNC, 4GAD, 5PharmGKB, 6HMDB, 7DrugBank, 8UniProtKB/TrEMBL, 9 Novoseek, and/or 10fRNAdb)
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    PubMed articles for LONP1 gene, integrated from 10 sources (see all 128):
    (articles sorted by number of sources associating them with LONP1)
        Utopia: connect your pdf to the dynamic
    world of online information

    1. Cloning and sequence analysis of cDNA for a human homolog of eubacterial ATP-dependent Lon proteases. (PubMed id 8119403)1, 2, 3, 9 Amerik A.Y.... Gorbalenya A.E. (FEBS Lett. 1994)
    2. A human mitochondrial ATP-dependent protease that is highly homologous to bacterial Lon protease. (PubMed id 8248235)1, 2, 3, 9 Wang N.... Maurizi M.R. (Proc. Natl. Acad. Sci. U.S.A. 1993)
    3. Roles for the human ATP-dependent Lon protease in mitochondrial DNA maintenance. (PubMed id 17420247)1, 2, 9 Lu B.... Suzuki C.K. (J. Biol. Chem. 2007)
    4. DNA and RNA binding by the mitochondrial lon protease is regulated by nucleotide and protein substrate. (PubMed id 14739292)1, 2, 9 Liu T.... Suzuki C.K. (J. Biol. Chem. 2004)
    5. Lon protease preferentially degrades oxidized mitochondrial aconitase by an ATP-stimulated mechanism. (PubMed id 12198491)1, 2, 9 Bota D.A. and Davies K.J. (Nat. Cell Biol. 2002)
    6. Synthesis, processing, and localization of human Lon protease. (PubMed id 7961901)1, 2, 9 Wang N.... Gottesman M.M. (J. Biol. Chem. 1994)
    7. The human LON protease binds to mitochondrial promoters in a single- stranded, site-specific, strand-specific manner. (PubMed id 9485316)1, 2, 9 Fu G.K. and Markovitz D.M. (Biochemistry 1998)
    8. Identification of rare DNA variants in mitochondrial disorders with improved array-based sequencing. (PubMed id 20843780)1, 2 Wang W.... Scharfe C. (Nucleic Acids Res. 2011)
    9. Genetic variants in nuclear-encoded mitochondrial genes influence AIDS progression. (PubMed id 20877624)1, 4 Hendrickson S.L....O'Brien S.J. (PLoS ONE 2010)
    10. The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). (PubMed id 15489334)1, 2 Gerhard D.S.... Malek J. (Genome Res. 2004)

    (in PubMed, OMIM, and NCBI Bookshelf)
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     ANDOR
    Aliases
    Disorders
    Free Text  

      Query String
    PubMed
    OMIM
    NCBI Bookshelf
      (Note: In FireFox, select the above section and copy using Ctrl-C)

    (According to Entrez Gene, HGNC, AceView, euGenes, Ensembl, miRBase, ECgene, Kegg, and/or H-InvDB)
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    Entrez Gene: 9361 HGNC: 9479 AceView: PRSS15 Ensembl:ENSG00000196365 euGenes: HUgn9361
    ECgene: LONP1 H-InvDB: LONP1

    (According to HUGE)
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      --

    (According to PharmGKB, ATLAS, HORDE, IMGT, LEIDEN, UniProtKB/Swiss-Prot, UniProtKB/TrEMBL, and/or others, e.g. Wikipedia and GeneReviews, via UniProtKB/Swiss-Prot)
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    NameDescription
    PharmGKB entry for LONP1 Pharmacogenomics, SNPs, Pathways

    (Patent information from GeneIP,
    Licensable technologies from WIS Yeda, Salk, Tufts,
    IP news from LifeMap Sciences, Inc.)
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    Patent Information for LONP1 gene:
    Search GeneIP for patents involving LONP1

    GeneCards and IP:
    Japan Patent Office Licenses GeneCards     European Patent Office Licenses GeneCards     Improving the IP Search



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