LOCUS       NM_006012               2410 bp    mRNA    linear   PRI 11-OCT-2024
DEFINITION  Homo sapiens caseinolytic mitochondrial matrix peptidase
            proteolytic subunit (CLPP), mRNA; nuclear gene for mitochondrial
            product.
ACCESSION   NM_006012
VERSION     NM_006012.4
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 2410)
  AUTHORS   Kou,X., Yang,X., Zhao,Z. and Li,L.
  TITLE     HSPA8-mediated stability of the CLPP protein regulates
            mitochondrial autophagy in cisplatin-resistant ovarian cancer cells
  JOURNAL   Acta Biochim Biophys Sin (Shanghai) 56 (3), 356-365 (2024)
   PUBMED   38419499
  REMARK    GeneRIF: HSPA8-mediated stability of the CLPP protein regulates
            mitochondrial autophagy in cisplatin-resistant ovarian cancer
            cells.
REFERENCE   2  (bases 1 to 2410)
  AUTHORS   Zhou,L.L., Zhang,T., Xue,Y., Yue,C., Pan,Y., Wang,P., Yang,T.,
            Li,M., Zhou,H., Ding,K., Gan,J., Ji,H. and Yang,C.G.
  TITLE     Selective activator of human ClpP triggers cell cycle arrest to
            inhibit lung squamous cell carcinoma
  JOURNAL   Nat Commun 14 (1), 7069 (2023)
   PUBMED   37923710
  REMARK    GeneRIF: Selective activator of human ClpP triggers cell cycle
            arrest to inhibit lung squamous cell carcinoma.
            Publication Status: Online-Only
REFERENCE   3  (bases 1 to 2410)
  AUTHORS   Wang,P., Zhang,T., Wang,X., Xiao,H., Li,H., Zhou,L.L., Yang,T.,
            Wei,B., Zhu,Z., Zhou,L., Yang,S., Lu,X., Zhang,Y., Huang,Y., Gan,J.
            and Yang,C.G.
  TITLE     Aberrant human ClpP activation disturbs mitochondrial proteome
            homeostasis to suppress pancreatic ductal adenocarcinoma
  JOURNAL   Cell Chem Biol 29 (9), 1396-1408 (2022)
   PUBMED   35905743
  REMARK    GeneRIF: Aberrant human ClpP activation disturbs mitochondrial
            proteome homeostasis to suppress pancreatic ductal adenocarcinoma.
REFERENCE   4  (bases 1 to 2410)
  AUTHORS   Rumyantseva,A., Popovic,M. and Trifunovic,A.
  TITLE     CLPP deficiency ameliorates neurodegeneration caused by impaired
            mitochondrial protein synthesis
  JOURNAL   Brain 145 (1), 92-104 (2022)
   PUBMED   35240691
  REMARK    GeneRIF: CLPP deficiency ameliorates neurodegeneration caused by
            impaired mitochondrial protein synthesis.
REFERENCE   5  (bases 1 to 2410)
  AUTHORS   Key,J., Torres-Odio,S., Bach,N.C., Gispert,S., Koepf,G.,
            Reichlmeir,M., West,A.P., Prokisch,H., Freisinger,P., Newman,W.G.,
            Shalev,S., Sieber,S.A., Wittig,I. and Auburger,G.
  TITLE     Inactivity of Peptidase ClpP Causes Primary Accumulation of
            Mitochondrial Disaggregase ClpX with Its Interacting Nucleoid
            Proteins, and of mtDNA
  JOURNAL   Cells 10 (12), 3354 (2021)
   PUBMED   34943861
  REMARK    GeneRIF: Inactivity of Peptidase ClpP Causes Primary Accumulation
            of Mitochondrial Disaggregase ClpX with Its Interacting Nucleoid
            Proteins, and of mtDNA.
            Publication Status: Online-Only
REFERENCE   6  (bases 1 to 2410)
  AUTHORS   Kang,S.G., Ortega,J., Singh,S.K., Wang,N., Huang,N.N., Steven,A.C.
            and Maurizi,M.R.
  TITLE     Functional proteolytic complexes of the human mitochondrial
            ATP-dependent protease, hClpXP
  JOURNAL   J Biol Chem 277 (23), 21095-21102 (2002)
   PUBMED   11923310
REFERENCE   7  (bases 1 to 2410)
  AUTHORS   de Sagarra,M.R., Mayo,I., Marco,S., Rodriguez-Vilarino,S.,
            Oliva,J., Carrascosa,J.L. and Casta n,J.G.
  TITLE     Mitochondrial localization and oligomeric structure of HClpP, the
            human homologue of E. coli ClpP
  JOURNAL   J Mol Biol 292 (4), 819-825 (1999)
   PUBMED   10525407
REFERENCE   8  (bases 1 to 2410)
  AUTHORS   Corydon,T.J., Bross,P., Holst,H.U., Neve,S., Kristiansen,K.,
            Gregersen,N. and Bolund,L.
  TITLE     A human homologue of Escherichia coli ClpP caseinolytic protease:
            recombinant expression, intracellular processing and subcellular
            localization
  JOURNAL   Biochem J 331 (Pt 1) (Pt 1), 309-316 (1998)
   PUBMED   9512494
REFERENCE   9  (bases 1 to 2410)
  AUTHORS   Bross,P., Andresen,B.S., Knudsen,I., Kruse,T.A. and Gregersen,N.
  TITLE     Human ClpP protease: cDNA sequence, tissue-specific expression and
            chromosomal assignment of the gene
  JOURNAL   FEBS Lett 377 (2), 249-252 (1995)
   PUBMED   8543061
REFERENCE   10 (bases 1 to 2410)
  AUTHORS   Newman,W.G., Friedman,T.B., Conway,G.S. and Demain,L.A.M.
  TITLE     Perrault Syndrome
  JOURNAL   (in) Adam MP, Feldman J, Mirzaa GM, Pagon RA, Wallace SE and
            Amemiya A (Eds.);
            GENEREVIEWS(R);
            (1993)
   PUBMED   25254289
COMMENT     REVIEWED REFSEQ: This record has been curated by NCBI staff. The
            reference sequence was derived from BC002956.1 and AC011491.7.
            This sequence is a reference standard in the RefSeqGene project.
            
            On May 14, 2019 this sequence version replaced NM_006012.3.
            
            Summary: The protein encoded by this gene belongs to the peptidase
            family S14 and hydrolyzes proteins into small peptides in the
            presence of ATP and magnesium. The protein is transported into
            mitochondrial matrix and is associated with the inner mitochondrial
            membrane. [provided by RefSeq, Jul 2008].
            
            Sequence Note: This RefSeq record was created from transcript and
            genomic sequence data to make the sequence consistent with the
            reference genome assembly. The genomic coordinates used for the
            transcript record were based on transcript alignments.
            
            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 :: SRR1803611.65598.1,
                                           DRR138522.66240.1 [ECO:0000332]
            RNAseq introns              :: single sample supports all introns
                                           SAMEA1965299, SAMEA1966682
                                           [ECO:0000348]
            ##Evidence-Data-END##
            
            ##RefSeq-Attributes-START##
            gene product(s) localized to mito. :: reported by MitoCarta
            MANE Ensembl match                 :: ENST00000245816.11/
                                                  ENSP00000245816.3
            RefSeq Select criteria             :: based on conservation,
                                                  expression
            ##RefSeq-Attributes-END##
            COMPLETENESS: full length.
PRIMARY     REFSEQ_SPAN         PRIMARY_IDENTIFIER PRIMARY_SPAN        COMP
            1-1070              BC002956.1         80-1149
            1071-2410           AC011491.7         70421-71760
FEATURES             Location/Qualifiers
     source          1..2410
                     /organism="Homo sapiens"
                     /mol_type="mRNA"
                     /db_xref="taxon:9606"
                     /chromosome="19"
                     /map="19p13.3"
     gene            1..2410
                     /gene="CLPP"
                     /gene_synonym="DFNB81; PRLTS3"
                     /note="caseinolytic mitochondrial matrix peptidase
                     proteolytic subunit"
                     /db_xref="GeneID:8192"
                     /db_xref="HGNC:HGNC:2084"
                     /db_xref="MIM:601119"
     exon            1..242
                     /gene="CLPP"
                     /gene_synonym="DFNB81; PRLTS3"
                     /inference="alignment:Splign:2.1.0"
     CDS             45..878
                     /gene="CLPP"
                     /gene_synonym="DFNB81; PRLTS3"
                     /EC_number="3.4.21.92"
                     /note="ATP-dependent protease ClpAP, proteolytic subunit,
                     human; putative ATP-dependent Clp protease proteolytic
                     subunit, mitochondrial; endopeptidase Clp; ClpP
                     caseinolytic protease, ATP-dependent, proteolytic subunit
                     homolog; ClpP caseinolytic peptidase ATP-dependent,
                     proteolytic subunit; ClpP caseinolytic peptidase,
                     ATP-dependent, proteolytic subunit homolog"
                     /codon_start=1
                     /product="ATP-dependent Clp protease proteolytic subunit,
                     mitochondrial precursor"
                     /protein_id="NP_006003.1"
                     /db_xref="CCDS:CCDS12162.1"
                     /db_xref="GeneID:8192"
                     /db_xref="HGNC:HGNC:2084"
                     /db_xref="MIM:601119"
                     /translation="MWPGILVGGARVASCRYPALGPRLAAHFPAQRPPQRTLQNGLAL
                     QRCLHATATRALPLIPIVVEQTGRGERAYDIYSRLLRERIVCVMGPIDDSVASLVIAQ
                     LLFLQSESNKKPIHMYINSPGGVVTAGLAIYDTMQYILNPICTWCVGQAASMGSLLLA
                     AGTPGMRHSLPNSRIMIHQPSGGARGQATDIAIQAEEIMKLKKQLYNIYAKHTKQSLQ
                     VIESAMERDRYMSPMEAQEFGILDKVLVHPPQDGEDEPTLVQKEPVEAAPAAEPVPAS
                     T"
     exon            243..314
                     /gene="CLPP"
                     /gene_synonym="DFNB81; PRLTS3"
                     /inference="alignment:Splign:2.1.0"
     exon            315..411
                     /gene="CLPP"
                     /gene_synonym="DFNB81; PRLTS3"
                     /inference="alignment:Splign:2.1.0"
     exon            412..599
                     /gene="CLPP"
                     /gene_synonym="DFNB81; PRLTS3"
                     /inference="alignment:Splign:2.1.0"
     exon            600..705
                     /gene="CLPP"
                     /gene_synonym="DFNB81; PRLTS3"
                     /inference="alignment:Splign:2.1.0"
     exon            706..2410
                     /gene="CLPP"
                     /gene_synonym="DFNB81; PRLTS3"
                     /inference="alignment:Splign:2.1.0"
     regulatory      1051..1056
                     /regulatory_class="polyA_signal_sequence"
                     /gene="CLPP"
                     /gene_synonym="DFNB81; PRLTS3"
                     /note="hexamer: ATTAAA"
     polyA_site      1072
                     /gene="CLPP"
                     /gene_synonym="DFNB81; PRLTS3"
                     /note="major polyA site"
     polyA_site      1107
                     /gene="CLPP"
                     /gene_synonym="DFNB81; PRLTS3"
     polyA_site      1209
                     /gene="CLPP"
                     /gene_synonym="DFNB81; PRLTS3"
     regulatory      1244..1249
                     /regulatory_class="polyA_signal_sequence"
                     /gene="CLPP"
                     /gene_synonym="DFNB81; PRLTS3"
                     /note="hexamer: AAGAAA"
     polyA_site      1261
                     /gene="CLPP"
                     /gene_synonym="DFNB81; PRLTS3"
     regulatory      1430..1435
                     /regulatory_class="polyA_signal_sequence"
                     /gene="CLPP"
                     /gene_synonym="DFNB81; PRLTS3"
                     /note="hexamer: AATACA"
     polyA_site      1448
                     /gene="CLPP"
                     /gene_synonym="DFNB81; PRLTS3"
     regulatory      2387..2392
                     /regulatory_class="polyA_signal_sequence"
                     /gene="CLPP"
                     /gene_synonym="DFNB81; PRLTS3"
                     /note="hexamer: AATAAA"
     polyA_site      2410
                     /gene="CLPP"
                     /gene_synonym="DFNB81; PRLTS3"
ORIGIN      
        1 gtagttccgc catcggacgg aagccgaccg gggcgtgcgg agggatgtgg cccggaatat
       61 tggtaggggg ggcccgggtg gcgtcatgca ggtaccccgc gctggggcct cgcctcgccg
      121 ctcactttcc agcgcagcgg ccgccgcagc ggacactcca gaacggcctg gccctgcagc
      181 ggtgcctgca cgcgacggcg acccgggctc tcccgctcat tcccatcgtg gtggagcaga
      241 cgggtcgcgg cgagcgcgcc tatgacatct actcgcggct gctgcgggag cgcatcgtgt
      301 gcgtcatggg cccgatcgat gacagcgttg ccagccttgt tatcgcacag ctcctcttcc
      361 tgcaatccga gagcaacaag aagcccatcc acatgtacat caacagccct ggtggtgtgg
      421 tgaccgcggg cctggccatc tacgacacga tgcagtacat cctcaacccg atctgcacct
      481 ggtgcgtggg ccaggccgcc agcatgggct ccctgcttct cgccgccggc accccaggca
      541 tgcgccactc gctccccaac tcccgtatca tgatccacca gccctcagga ggcgcccggg
      601 gccaagccac agacattgcc atccaggcag aggagatcat gaagctcaag aagcagctct
      661 ataacatcta cgccaagcac accaaacaga gcctgcaggt gatcgagtcc gccatggaga
      721 gggaccgcta catgagcccc atggaggccc aggagtttgg catcttagac aaggttctgg
      781 tccaccctcc ccaggacggt gaggatgagc ccacgctggt gcagaaggag cctgtagaag
      841 cagcgccggc agcagaacct gtcccagcta gcacctgaga gctgggcctc ctctccagaa
      901 tcatgtggag gggccagagg cctgccagac ccccagctgg gccctgctca ccccttgttg
      961 ctgggcttgg aggggcctct tgaggaactt ttaatttgca ggggtgcccg ctatggacgg
     1021 ggcattccag ctgagacact gtgattttaa attaaatctt tgtggtcttt gctctgcgtc
     1081 tgggacaccc tcccttctgc accatgacag cgtgtacaca tctgtgtttc acaggcccct
     1141 tctgcttttg tcctgacccc aagaggcaag ccatgtcttc cttcaacaga ttctcctgat
     1201 catatcctat attccaggca gtgttctttt gtgaagaaga cacaagaaac cctcctcaaa
     1261 agagctccat tcttgtggca gtagacagtt actaaaaaaa acaaaacagg ccaggcgcgc
     1321 tggctcacgc ctgtaatccc agcactttgg gagcccgagg caggcggatc atgaggtcag
     1381 gagatcgaga acatcctggc taacatggtg aaaccccatt tctactaaaa atacaaaaat
     1441 tagccgagcg tggtgcacct gtaatcccag ctactccgga ggctgaggca gaagaattgc
     1501 ttgaacttag gaggtggagg ttgcagtgag caggatcacg ccactgccct ttagcctggg
     1561 caacagagca aggctctgtc tcaaaaaaaa aaaaaaacaa aaacaggaaa ggtggcatgc
     1621 aagtgtgagg gtaagcgtgt tacagttttt tatggggtaa tcagggaagc cctcactgag
     1681 aaggtggtgg ttgagcaaag cctcaaagga ggtgaggggt ggagcctgtg tccaggcaga
     1741 gggcacagcc agtgcaaagg ccctgtgaca ggactgtacc tggcatgtga ggacctatga
     1801 ggaggcccgt ggctgaggga aggtggaaga gaggagatca aaaagtagag gtgaaaatta
     1861 gccaggcgtg atggtgcact tgaacccggg aggcagaggt tgcagtgagc caagatcacg
     1921 ccactgcact ccagcctggg caacaaagcg agattctgtc tccaaaaaaa aaaaagtgga
     1981 ggtgattgag aatagatcat aaaaggcctg acatctaaaa ggagggatgt gaaccatgga
     2041 gggttttgag aagaggaagg aaaataagga tgactatgac tcctgaggag tgcaccatga
     2101 gcagaaactg gagagcagtt aggatagttg gagtaatcta gacgaaagct gatggcagcc
     2161 ctggccgagt gatgagaaaa agtcagattc tagctaggtg ggatgtgaca gagagcagtt
     2221 tccagaaact ccgagctgat ggcccaaagc aggaagatgg agacctgtcg cctgagatgg
     2281 gaagtgtagg caaaggaaca ggcttgggga gggatcagga gttgattttg gatatattat
     2341 acctgatgtt ttgatatgtc cctcgacatc agggtcaaga ggttgaaata aaaacgtagg
     2401 tgtcatcggc
//
