LOCUS       NM_004793               3092 bp    mRNA    linear   PRI 17-SEP-2024
DEFINITION  Homo sapiens lon peptidase 1, mitochondrial (LONP1), transcript
            variant 1, mRNA; nuclear gene for mitochondrial product.
ACCESSION   NM_004793
VERSION     NM_004793.4
KEYWORDS    RefSeq; MANE Select.
SOURCE      Homo sapiens (human)
  ORGANISM  Homo sapiens
            Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi;
            Mammalia; Eutheria; Euarchontoglires; Primates; Haplorrhini;
            Catarrhini; Hominidae; Homo.
REFERENCE   1  (bases 1 to 3092)
  AUTHORS   Zhang,C., Shen,S., Xu,L., Li,M., Tian,B., Yao,L. and Zhu,X.
  TITLE     LONP1 alleviates ageing-related renal fibrosis by maintaining
            mitochondrial homeostasis
  JOURNAL   J Cell Mol Med 28 (17), e70090 (2024)
   PUBMED   39261902
  REMARK    GeneRIF: LONP1 alleviates ageing-related renal fibrosis by
            maintaining mitochondrial homeostasis.
REFERENCE   2  (bases 1 to 3092)
  AUTHORS   Wang,W., Ma,X., Bhatta,S., Shao,C., Zhao,F., Fujioka,H., Torres,S.,
            Wu,F. and Zhu,X.
  TITLE     Intraneuronal beta-amyloid impaired mitochondrial proteostasis
            through the impact on LONP1
  JOURNAL   Proc Natl Acad Sci U S A 120 (51), e2316823120 (2023)
   PUBMED   38091289
  REMARK    GeneRIF: Intraneuronal beta-amyloid impaired mitochondrial
            proteostasis through the impact on LONP1.
REFERENCE   3  (bases 1 to 3092)
  AUTHORS   Shan,S., Liu,Z., Wang,S., Liu,Z., Chao,S., Zhang,C., Li,M. and
            Song,F.
  TITLE     Mitochondrial oxidative stress regulates LonP1-TDP-43 pathway and
            rises mitochondrial damage in carbon tetrachloride-induced liver
            fibrosis
  JOURNAL   Ecotoxicol Environ Saf 264, 115409 (2023)
   PUBMED   37647804
  REMARK    GeneRIF: Mitochondrial oxidative stress regulates LonP1-TDP-43
            pathway and rises mitochondrial damage in carbon
            tetrachloride-induced liver fibrosis.
REFERENCE   4  (bases 1 to 3092)
  AUTHORS   Sekine,Y., Houston,R., Eckl,E.M., Fessler,E., Narendra,D.P.,
            Jae,L.T. and Sekine,S.
  TITLE     A mitochondrial iron-responsive pathway regulated by DELE1
  JOURNAL   Mol Cell 83 (12), 2059-2076 (2023)
   PUBMED   37327776
REFERENCE   5  (bases 1 to 3092)
  AUTHORS   Wu,L., Yan,X., Sun,R., Ma,Y., Yao,W., Gao,B., Zhang,Q., You,J.,
            Wang,H., Han,Q. and Sun,X.
  TITLE     Sirt3 restricts tumor initiation via promoting LONP1 deacetylation
            and K63 ubiquitination
  JOURNAL   J Transl Med 21 (1), 81 (2023)
   PUBMED   36739437
  REMARK    GeneRIF: Sirt3 restricts tumor initiation via promoting LONP1
            deacetylation and K63 ubiquitination.
            Publication Status: Online-Only
REFERENCE   6  (bases 1 to 3092)
  AUTHORS   Fu,G.K. and Markovitz,D.M.
  TITLE     The human LON protease binds to mitochondrial promoters in a
            single-stranded, site-specific, strand-specific manner
  JOURNAL   Biochemistry 37 (7), 1905-1909 (1998)
   PUBMED   9485316
REFERENCE   7  (bases 1 to 3092)
  AUTHORS   Korenberg,J.R., Chen,X.N., Adams,M.D. and Venter,J.C.
  TITLE     Toward a cDNA map of the human genome
  JOURNAL   Genomics 29 (2), 364-370 (1995)
   PUBMED   8666383
REFERENCE   8  (bases 1 to 3092)
  AUTHORS   Wang,N., Maurizi,M.R., Emmert-Buck,L. and Gottesman,M.M.
  TITLE     Synthesis, processing, and localization of human Lon protease
  JOURNAL   J Biol Chem 269 (46), 29308-29313 (1994)
   PUBMED   7961901
REFERENCE   9  (bases 1 to 3092)
  AUTHORS   Amerik,A.Yu., Petukhova,G.V., Grigorenko,V.G., Lykov,I.P.,
            Yarovoi,S.V., Lipkin,V.M. and Gorbalenya,A.E.
  TITLE     Cloning and sequence analysis of cDNA for a human homolog of
            eubacterial ATP-dependent Lon proteases
  JOURNAL   FEBS Lett 340 (1-2), 25-28 (1994)
   PUBMED   8119403
REFERENCE   10 (bases 1 to 3092)
  AUTHORS   Wang,N., Gottesman,S., Willingham,M.C., Gottesman,M.M. and
            Maurizi,M.R.
  TITLE     A human mitochondrial ATP-dependent protease that is highly
            homologous to bacterial Lon protease
  JOURNAL   Proc Natl Acad Sci U S A 90 (23), 11247-11251 (1993)
   PUBMED   8248235
COMMENT     REVIEWED REFSEQ: This record has been curated by NCBI staff. The
            reference sequence was derived from BI906625.1 and BC000235.1.
            This sequence is a reference standard in the RefSeqGene project.
            
            On May 17, 2019 this sequence version replaced NM_004793.3.
            
            Summary: 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].
            
            Transcript Variant: This variant (1) encodes the longest isoform
            (1).
            
            Publication Note:  This RefSeq record includes a subset of the
            publications that are available for this gene. Please see the Gene
            record to access additional publications.
            
            ##Evidence-Data-START##
            Transcript exon combination :: BC000235.1, SRR1660803.30729.1
                                           [ECO:0000332]
            RNAseq introns              :: mixed sample support SAMEA1965299,
                                           SAMEA1966682 [ECO:0006172]
            ##Evidence-Data-END##
            
            ##RefSeq-Attributes-START##
            gene product(s) localized to mito. :: reported by MitoCarta
            MANE Ensembl match                 :: ENST00000360614.8/
                                                  ENSP00000353826.2
            RefSeq Select criteria             :: based on conservation,
                                                  expression, longest protein
            ##RefSeq-Attributes-END##
            COMPLETENESS: complete on the 3' end.
PRIMARY     REFSEQ_SPAN         PRIMARY_IDENTIFIER PRIMARY_SPAN        COMP
            1-9                 BI906625.1         144-152
            10-3092             BC000235.1         1-3083
FEATURES             Location/Qualifiers
     source          1..3092
                     /organism="Homo sapiens"
                     /mol_type="mRNA"
                     /db_xref="taxon:9606"
                     /chromosome="19"
                     /map="19p13.3"
     gene            1..3092
                     /gene="LONP1"
                     /gene_synonym="CODASS; hLON; LON; LonHS; LONP; PIM1;
                     PRSS15"
                     /note="lon peptidase 1, mitochondrial"
                     /db_xref="GeneID:9361"
                     /db_xref="HGNC:HGNC:9479"
                     /db_xref="MIM:605490"
     exon            1..443
                     /gene="LONP1"
                     /gene_synonym="CODASS; hLON; LON; LonHS; LONP; PIM1;
                     PRSS15"
                     /inference="alignment:Splign:2.1.0"
     CDS             15..2894
                     /gene="LONP1"
                     /gene_synonym="CODASS; hLON; LON; LonHS; LONP; PIM1;
                     PRSS15"
                     /EC_number="3.4.21.53"
                     /note="isoform 1 precursor is encoded by transcript
                     variant 1; hLON ATP-dependent protease; lon protease
                     homolog, mitochondrial; mitochondrial lon protease-like
                     protein; serine protease 15; mitochondrial ATP-dependent
                     protease Lon"
                     /codon_start=1
                     /product="lon protease homolog, mitochondrial isoform 1
                     precursor"
                     /protein_id="NP_004784.2"
                     /db_xref="CCDS:CCDS12148.1"
                     /db_xref="GeneID:9361"
                     /db_xref="HGNC:HGNC:9479"
                     /db_xref="MIM:605490"
                     /translation="MAASTGYVRLWGAARCWVLRRPMLAAAGGRVPTAAGAWLLRGQR
                     TCDASPPWALWGRGPAIGGQWRGFWEASSRGGGAFSGGEDASEGGAEEGAGGAGGSAG
                     AGEGPVITALTPMTIPDVFPHLPLIAITRNPVFPRFIKIIEVKNKKLVELLRRKVRLA
                     QPYVGVFLKRDDSNESDVVESLDEIYHTGTFAQIHEMQDLGDKLRMIVMGHRRVHISR
                     QLEVEPEEPEAENKHKPRRKSKRGKKEAEDELSARHPAELAMEPTPELPAEVLMVEVE
                     NVVHEDFQVTEEVKALTAEIVKTIRDIIALNPLYRESVLQMMQAGQRVVDNPIYLSDM
                     GAALTGAESHELQDVLEETNIPKRLYKALSLLKKEFELSKLQQRLGREVEEKIKQTHR
                     KYLLQEQLKIIKKELGLEKDDKDAIEEKFRERLKELVVPKHVMDVVDEELSKLGLLDN
                     HSSEFNVTRNYLDWLTSIPWGKYSNENLDLARAQAVLEEDHYGMEDVKKRILEFIAVS
                     QLRGSTQGKILCFYGPPGVGKTSIARSIARALNREYFRFSVGGMTDVAEIKGHRRTYV
                     GAMPGKIIQCLKKTKTENPLILIDEVDKIGRGYQGDPSSALLELLDPEQNANFLDHYL
                     DVPVDLSKVLFICTANVTDTIPEPLRDRMEMINVSGYVAQEKLAIAERYLVPQARALC
                     GLDESKAKLSSDVLTLLIKQYCRESGVRNLQKQVEKVLRKSAYKIVSGEAESVEVTPE
                     NLQDFVGKPVFTVERMYDVTPPGVVMGLAWTAMGGSTLFVETSLRRPQDKDAKGDKDG
                     SLEVTGQLGEVMKESARIAYTFARAFLMQHAPANDYLVTSHIHLHVPEGATPKDGPSA
                     GCTIVTALLSLAMGRPVRQNLAMTGEVSLTGKILPVGGIKEKTIAAKRAGVTCIVLPA
                     ENKKDFYDLAAFITEGLEVHFVEHYREIFDIAFPDEQAEALAVER"
     exon            444..532
                     /gene="LONP1"
                     /gene_synonym="CODASS; hLON; LON; LonHS; LONP; PIM1;
                     PRSS15"
                     /inference="alignment:Splign:2.1.0"
     exon            533..652
                     /gene="LONP1"
                     /gene_synonym="CODASS; hLON; LON; LonHS; LONP; PIM1;
                     PRSS15"
                     /inference="alignment:Splign:2.1.0"
     exon            653..884
                     /gene="LONP1"
                     /gene_synonym="CODASS; hLON; LON; LonHS; LONP; PIM1;
                     PRSS15"
                     /inference="alignment:Splign:2.1.0"
     exon            885..946
                     /gene="LONP1"
                     /gene_synonym="CODASS; hLON; LON; LonHS; LONP; PIM1;
                     PRSS15"
                     /inference="alignment:Splign:2.1.0"
     exon            947..1076
                     /gene="LONP1"
                     /gene_synonym="CODASS; hLON; LON; LonHS; LONP; PIM1;
                     PRSS15"
                     /inference="alignment:Splign:2.1.0"
     exon            1077..1160
                     /gene="LONP1"
                     /gene_synonym="CODASS; hLON; LON; LonHS; LONP; PIM1;
                     PRSS15"
                     /inference="alignment:Splign:2.1.0"
     exon            1161..1381
                     /gene="LONP1"
                     /gene_synonym="CODASS; hLON; LON; LonHS; LONP; PIM1;
                     PRSS15"
                     /inference="alignment:Splign:2.1.0"
     exon            1382..1520
                     /gene="LONP1"
                     /gene_synonym="CODASS; hLON; LON; LonHS; LONP; PIM1;
                     PRSS15"
                     /inference="alignment:Splign:2.1.0"
     exon            1521..1699
                     /gene="LONP1"
                     /gene_synonym="CODASS; hLON; LON; LonHS; LONP; PIM1;
                     PRSS15"
                     /inference="alignment:Splign:2.1.0"
     exon            1700..1787
                     /gene="LONP1"
                     /gene_synonym="CODASS; hLON; LON; LonHS; LONP; PIM1;
                     PRSS15"
                     /inference="alignment:Splign:2.1.0"
     exon            1788..1910
                     /gene="LONP1"
                     /gene_synonym="CODASS; hLON; LON; LonHS; LONP; PIM1;
                     PRSS15"
                     /inference="alignment:Splign:2.1.0"
     exon            1911..2027
                     /gene="LONP1"
                     /gene_synonym="CODASS; hLON; LON; LonHS; LONP; PIM1;
                     PRSS15"
                     /inference="alignment:Splign:2.1.0"
     exon            2028..2168
                     /gene="LONP1"
                     /gene_synonym="CODASS; hLON; LON; LonHS; LONP; PIM1;
                     PRSS15"
                     /inference="alignment:Splign:2.1.0"
     exon            2169..2334
                     /gene="LONP1"
                     /gene_synonym="CODASS; hLON; LON; LonHS; LONP; PIM1;
                     PRSS15"
                     /inference="alignment:Splign:2.1.0"
     exon            2335..2552
                     /gene="LONP1"
                     /gene_synonym="CODASS; hLON; LON; LonHS; LONP; PIM1;
                     PRSS15"
                     /inference="alignment:Splign:2.1.0"
     exon            2553..2717
                     /gene="LONP1"
                     /gene_synonym="CODASS; hLON; LON; LonHS; LONP; PIM1;
                     PRSS15"
                     /inference="alignment:Splign:2.1.0"
     exon            2718..3092
                     /gene="LONP1"
                     /gene_synonym="CODASS; hLON; LON; LonHS; LONP; PIM1;
                     PRSS15"
                     /inference="alignment:Splign:2.1.0"
     regulatory      3074..3079
                     /regulatory_class="polyA_signal_sequence"
                     /gene="LONP1"
                     /gene_synonym="CODASS; hLON; LON; LonHS; LONP; PIM1;
                     PRSS15"
                     /note="hexamer: ATTAAA"
     polyA_site      3092
                     /gene="LONP1"
                     /gene_synonym="CODASS; hLON; LON; LonHS; LONP; PIM1;
                     PRSS15"
                     /note="major polyA site"
ORIGIN      
        1 agtatggccg ggctatggcg gcgagcactg gctacgtgcg actgtgggga gcggcgcggt
       61 gctgggtgct gcggcggccg atgctggccg ccgccggggg gcgggttccc actgcagcag
      121 gagcgtggtt gctccgaggc cagcggacct gcgacgcctc tcctccttgg gcactgtggg
      181 gccgaggccc ggcaattggg ggccaatggc gggggttttg ggaagcgagc agccgcggcg
      241 gaggcgcatt ctcggggggc gaggacgcct ccgagggcgg cgcggaggaa ggagccggcg
      301 gcgcgggggg cagcgcgggc gccggggaag gcccggtcat aacggcgctc acgcccatga
      361 cgatccccga tgtgtttccg cacctgccgc tcatcgccat cacccgcaac ccggtgttcc
      421 cgcgctttat caagattatc gaggttaaaa ataagaagtt ggttgagctg ctgagaagga
      481 aagttcgtct cgcccagcct tatgtcggcg tctttctaaa gagagatgac agcaatgagt
      541 cggatgtggt cgagagcctg gatgaaatct accacacggg gacgtttgcc cagatccatg
      601 agatgcagga ccttggggac aagctgcgca tgatcgtcat gggacacaga agagtccata
      661 tcagcagaca gctggaggtg gagcccgagg agccggaggc ggagaacaag cacaagcccc
      721 gcaggaagtc aaagcggggc aagaaggagg cggaggacga gctgagcgcc aggcacccgg
      781 cggagctggc gatggagccc acccctgagc tcccggctga ggtgctcatg gtggaggtag
      841 agaacgttgt ccacgaggac ttccaggtca cggaggaggt gaaagccctg actgcagaga
      901 tcgtgaagac catccgggac atcattgcct tgaaccctct ctacagggag tcagtgctgc
      961 agatgatgca ggctggccag cgggtggtgg acaaccccat ctacctgagc gacatgggcg
     1021 ccgcgctcac cggggccgag tcccatgagc tgcaggacgt cctggaagag accaatattc
     1081 ctaagcggct gtacaaggcc ctctccctgc tgaagaagga atttgaactg agcaagctgc
     1141 agcagcgcct ggggcgggag gtggaggaga agatcaagca gacccaccgt aagtacctgc
     1201 tgcaggagca gctaaagatc atcaagaagg agctgggcct ggagaaggac gacaaggatg
     1261 ccatcgagga gaagttccgg gagcgcctga aggagctcgt ggtccccaag cacgtcatgg
     1321 atgttgtgga cgaggagctg agcaagctgg gcctgctgga caaccactcc tcggagttca
     1381 atgtcacccg caactaccta gactggctca cgtccatccc ttggggcaag tacagcaacg
     1441 agaacctgga cctggcgcgg gcacaggcag tgctggagga agaccactac ggcatggagg
     1501 acgtcaagaa acgcatcctg gagttcattg ccgttagcca gctccgcggc tccacccagg
     1561 gcaagatcct ctgcttctat ggcccccctg gcgtgggtaa gaccagcatt gctcgctcca
     1621 tcgcccgcgc cctgaaccga gagtacttcc gcttcagcgt cgggggcatg actgacgtgg
     1681 ctgagatcaa gggccacagg cggacctacg tgggcgccat gcccgggaag atcatccagt
     1741 gtttgaagaa gaccaagacg gagaaccccc tgatcctcat cgacgaggtg gacaagatcg
     1801 gccgaggcta ccagggggac ccgtcgtcgg cactgctgga gctgctggac ccagagcaga
     1861 atgccaactt cctggaccac tacctggacg tgcccgtgga cttgtccaag gtgctgttca
     1921 tctgcacggc caacgtcacg gacaccatcc ccgagccgct gcgagaccgt atggagatga
     1981 tcaacgtgtc gggctacgtg gcccaggaga agctggccat tgcggagcgc tacctggtgc
     2041 cccaggctcg cgccctgtgt ggcttggatg agagcaaggc caagctgtca tcggacgtgc
     2101 tgacgctgct catcaagcag tactgccgcg agagcggtgt ccgcaacctg cagaagcaag
     2161 tggagaaggt gttacggaaa tcggcctaca agattgtcag cggcgaggcc gagtccgtgg
     2221 aggtgacgcc cgagaacctg caggacttcg tggggaagcc cgtgttcacc gtggagcgca
     2281 tgtatgacgt gacaccgccc ggcgtggtca tggggctggc ctggaccgca atgggaggct
     2341 ccacgctgtt tgtggagaca tccctgagac ggccacagga caaggatgcc aagggtgaca
     2401 aggatggcag cctggaggtg acaggccagc tgggggaggt gatgaaggag agcgcccgca
     2461 tagcctacac cttcgccaga gccttcctca tgcagcacgc ccccgccaat gactacctgg
     2521 tgacctcaca catccacctg catgtgcccg agggcgccac ccccaaggac ggcccaagcg
     2581 caggctgcac catcgtcacg gccctgctgt ccctggccat gggcaggcct gtccggcaga
     2641 atctggccat gactggcgaa gtctccctca cgggcaagat cctgcctgtt ggtggcatca
     2701 aggagaagac cattgcggcc aagcgcgcag gggtgacgtg catcgtcctg ccagccgaga
     2761 acaagaagga cttctacgac ctggcagcct tcatcaccga gggcctggag gtgcacttcg
     2821 tggaacacta ccgggagatc ttcgacatcg ccttcccgga cgagcaggca gaggcgctgg
     2881 ccgtggaacg gtgacggcca ccccgggact gcaggcggcg gatgtcaggc cctgtctggg
     2941 ccagaactga gcgctgtggg gagcgcgccc ggacctggca gtggagccac cgagcgagca
     3001 gctcggtcca gtgacccaga tcccagggac ctcagtcggc ttaatcagag tgtggcatag
     3061 aagctattta atgattaaag tcatttgcag ta
//
