LOCUS       NM_009906               3573 bp    mRNA    linear   ROD 09-FEB-2025
DEFINITION  Mus musculus tripeptidyl peptidase I (Tpp1), mRNA.
ACCESSION   NM_009906
VERSION     NM_009906.6
KEYWORDS    RefSeq; RefSeq Select.
SOURCE      Mus musculus (house mouse)
  ORGANISM  Mus musculus
            Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi;
            Mammalia; Eutheria; Euarchontoglires; Glires; Rodentia; Myomorpha;
            Muroidea; Muridae; Murinae; Mus; Mus.
REFERENCE   1  (bases 1 to 3573)
  AUTHORS   Kovacs,A.D., Gonzalez Hernandez,J.L. and Pearce,D.A.
  TITLE     Acidified drinking water improves motor function, prevents tremors
            and changes disease trajectory in Cln2R207X mice, a model of late
            infantile Batten disease
  JOURNAL   Sci Rep 13 (1), 19229 (2023)
   PUBMED   37932327
  REMARK    Publication Status: Online-Only
REFERENCE   2  (bases 1 to 3573)
  AUTHORS   Sleat,D.E., Banach-Petrosky,W., Larrimore,K.E., Nemtsova,Y.,
            Wiseman,J.A., Najafi,A., Johnson,D., Poole,T.A., Takahashi,K.,
            Cooper,J.D. and Lobel,P.
  TITLE     A mouse mutant deficient in both neuronal ceroid
            lipofuscinosis-associated proteins CLN3 and TPP1
  JOURNAL   J Inherit Metab Dis 46 (4), 720-734 (2023)
   PUBMED   37078466
  REMARK    GeneRIF: A mouse mutant deficient in both neuronal ceroid
            lipofuscinosis-associated proteins CLN3 and TPP1.
REFERENCE   3  (bases 1 to 3573)
  AUTHORS   Takahashi,K., Eultgen,E.M., Wang,S.H., Rensing,N.R., Nelvagal,H.R.,
            Dearborn,J.T., Danos,O., Buss,N., Sands,M.S., Wong,M. and
            Cooper,J.D.
  TITLE     Gene therapy ameliorates spontaneous seizures associated with
            cortical neuron loss in a Cln2R207X mouse model
  JOURNAL   J Clin Invest 133 (12), e165908 (2023)
   PUBMED   37104037
  REMARK    Publication Status: Online-Only
REFERENCE   4  (bases 1 to 3573)
  AUTHORS   El-Hage,N., Haney,M.J., Zhao,Y., Rodriguez,M., Wu,Z., Liu,M.,
            Swain,C.J., Yuan,H. and Batrakova,E.V.
  TITLE     Extracellular Vesicles Released by Genetically Modified Macrophages
            Activate Autophagy and Produce Potent Neuroprotection in Mouse
            Model of Lysosomal Storage Disorder, Batten Disease
  JOURNAL   Cells 12 (11), 1497 (2023)
   PUBMED   37296618
  REMARK    Publication Status: Online-Only
REFERENCE   5  (bases 1 to 3573)
  AUTHORS   Dutta,D., Paidi,R.K., Raha,S., Roy,A., Chandra,S. and Pahan,K.
  TITLE     Treadmill exercise reduces alpha-synuclein spreading via PPARalpha
  JOURNAL   Cell Rep 40 (2), 111058 (2022)
   PUBMED   35830804
  REMARK    Erratum:[Cell Rep. 2024 Aug 27;43(8):114546. doi:
            10.1016/j.celrep.2024.114546.. PMID: 39024097]
REFERENCE   6  (bases 1 to 3573)
  AUTHORS   Kannan,K., Kaminski,N., Rechavi,G., Jakob-Hirsch,J., Amariglio,N.
            and Givol,D.
  TITLE     DNA microarray analysis of genes involved in p53 mediated
            apoptosis: activation of Apaf-1
  JOURNAL   Oncogene 20 (26), 3449-3455 (2001)
   PUBMED   11423996
REFERENCE   7  (bases 1 to 3573)
  AUTHORS   Katz,M.L., Liu,P.C., Grob-Nunn,S.E., Shibuya,H. and Johnson,G.S.
  TITLE     Characterization and chromosomal mapping of a mouse ortholog of the
            late-infantile ceroid-lipofuscinosis gene CLN2
  JOURNAL   Mamm Genome 10 (11), 1050-1053 (1999)
   PUBMED   10556422
REFERENCE   8  (bases 1 to 3573)
  AUTHORS   Mitchison,H.M., Bernard,D.J., Greene,N.D., Cooper,J.D.,
            Junaid,M.A., Pullarkat,R.K., de Vos,N., Breuning,M.H., Owens,J.W.,
            Mobley,W.C., Gardiner,R.M., Lake,B.D., Taschner,P.E. and
            Nussbaum,R.L.
  TITLE     Targeted disruption of the Cln3 gene provides a mouse model for
            Batten disease. The Batten Mouse Model Consortium [corrected]
  JOURNAL   Neurobiol Dis 6 (5), 321-334 (1999)
   PUBMED   10527801
  REMARK    Erratum:[Neurobiol Dis 2000 Apr;7(2):127]
REFERENCE   9  (bases 1 to 3573)
  AUTHORS   Sleat,D.E., Gin,R.M., Sohar,I., Wisniewski,K., Sklower-Brooks,S.,
            Pullarkat,R.K., Palmer,D.N., Lerner,T.J., Boustany,R.M., Uldall,P.,
            Siakotos,A.N., Donnelly,R.J. and Lobel,P.
  TITLE     Mutational analysis of the defective protease in classic
            late-infantile neuronal ceroid lipofuscinosis, a neurodegenerative
            lysosomal storage disorder
  JOURNAL   Am J Hum Genet 64 (6), 1511-1523 (1999)
   PUBMED   10330339
  REMARK    Erratum:[Am J Hum Genet. 2004 Dec;75(6):1158]
REFERENCE   10 (bases 1 to 3573)
  AUTHORS   Vines,D.J. and Warburton,M.J.
  TITLE     Classical late infantile neuronal ceroid lipofuscinosis fibroblasts
            are deficient in lysosomal tripeptidyl peptidase I
  JOURNAL   FEBS Lett 443 (2), 131-135 (1999)
   PUBMED   9989590
COMMENT     REVIEWED REFSEQ: This record has been curated by NCBI staff. The
            reference sequence was derived from AC121823.3, AK002418.1,
            AK048279.1 and AA499658.1.
            
            On Nov 11, 2015 this sequence version replaced NM_009906.5.
            
            Summary: This gene encodes a lysosomal serine protease that cleaves
            N-terminal tripeptides from protein substrates. The encoded
            preproprotein undergoes autocatalytic processing to generate a
            mature enzyme. Mice lacking the encoded protein exhibit a
            progressive neurodegeneration and a greatly shortened lifespan. At
            the cellular level, mice lacking the encoded protein exhibit
            accumulation of autofluorescent lipopigments. Mutations in the
            human ortholog of this gene cause classical late-infantile neuronal
            ceroid lipofuscinosis. [provided by RefSeq, Nov 2015].
            
            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 :: AK048279.1, AK170781.1 [ECO:0000332]
            RNAseq introns              :: single sample supports all introns
                                           SAMN01164131, SAMN01164134
                                           [ECO:0000348]
            ##Evidence-Data-END##
            
            ##RefSeq-Attributes-START##
            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
            1-10                AC121823.3         114333-114342
            11-2290             AK002418.1         2-2281
            2291-2301           AK048279.1         2264-2274
            2302-2302           AC121823.3         120501-120501
            2303-3522           AK048279.1         2275-3494
            3523-3573           AA499658.1         461-511
FEATURES             Location/Qualifiers
     source          1..3573
                     /organism="Mus musculus"
                     /mol_type="mRNA"
                     /strain="C57BL/6"
                     /db_xref="taxon:10090"
                     /chromosome="7"
                     /map="7 55.97 cM"
     gene            1..3573
                     /gene="Tpp1"
                     /gene_synonym="Cln2; LPIC; TPP-1; TPP-I"
                     /note="tripeptidyl peptidase I"
                     /db_xref="GeneID:12751"
                     /db_xref="MGI:MGI:1336194"
     exon            1..65
                     /gene="Tpp1"
                     /gene_synonym="Cln2; LPIC; TPP-1; TPP-I"
                     /inference="alignment:Splign:2.1.0"
     CDS             49..1737
                     /gene="Tpp1"
                     /gene_synonym="Cln2; LPIC; TPP-1; TPP-I"
                     /EC_number="3.4.14.9"
                     /note="tripeptidyl-peptidase 1; tripeptidyl
                     aminopeptidase; lysosomal pepstatin-insensitive protease;
                     ceroid-lipofuscinosis, neuronal 2"
                     /codon_start=1
                     /product="tripeptidyl-peptidase 1 preproprotein"
                     /protein_id="NP_034036.1"
                     /db_xref="CCDS:CCDS21661.1"
                     /db_xref="GeneID:12751"
                     /db_xref="MGI:MGI:1336194"
                     /translation="MGLQARLLGLLALVIAGKCTYNPEPDQRWMLPPGWVSLGRVDPE
                     EELSLTFALKQRNLERLSELVQAVSDPSSPQYGKYLTLEDVAELVQPSPLTLLTVQKW
                     LSAAGARNCDSVTTQDFLTCWLSVRQAELLLPGAEFHRYVGGPTKTHVIRSPHPYQLP
                     QALAPHVDFVGGLHRFPPSSPRQRPEPQQVGTVSLHLGVTPSVLRQRYNLTAKDVGSG
                     TTNNSQACAQFLEQYFHNSDLTEFMRLFGGSFTHQASVAKVVGKQGRGRAGIEASLDV
                     EYLMSAGANISTWVYSSPGRHEAQEPFLQWLLLLSNESSLPHVHTVSYGDDEDSLSSI
                     YIQRVNTEFMKAAARGLTLLFASGDTGAGCWSVSGRHKFRPSFPASSPYVTTVGGTSF
                     KNPFLITDEVVDYISGGGFSNVFPRPPYQEEAVAQFLKSSSHLPPSSYFNASGRAYPD
                     VAALSDGYWVVSNMVPIPWVSGTSASTPVFGGILSLINEHRILNGRPPLGFLNPRLYQ
                     QHGTGLFDVTHGCHESCLNEEVEGQGFCSGPGWDPVTGWGTPNFPALLKTLLNP"
     exon            66..137
                     /gene="Tpp1"
                     /gene_synonym="Cln2; LPIC; TPP-1; TPP-I"
                     /inference="alignment:Splign:2.1.0"
     exon            138..277
                     /gene="Tpp1"
                     /gene_synonym="Cln2; LPIC; TPP-1; TPP-I"
                     /inference="alignment:Splign:2.1.0"
     exon            278..428
                     /gene="Tpp1"
                     /gene_synonym="Cln2; LPIC; TPP-1; TPP-I"
                     /inference="alignment:Splign:2.1.0"
     exon            429..556
                     /gene="Tpp1"
                     /gene_synonym="Cln2; LPIC; TPP-1; TPP-I"
                     /inference="alignment:Splign:2.1.0"
     exon            557..732
                     /gene="Tpp1"
                     /gene_synonym="Cln2; LPIC; TPP-1; TPP-I"
                     /inference="alignment:Splign:2.1.0"
     exon            733..931
                     /gene="Tpp1"
                     /gene_synonym="Cln2; LPIC; TPP-1; TPP-I"
                     /inference="alignment:Splign:2.1.0"
     exon            932..1120
                     /gene="Tpp1"
                     /gene_synonym="Cln2; LPIC; TPP-1; TPP-I"
                     /inference="alignment:Splign:2.1.0"
     exon            1121..1190
                     /gene="Tpp1"
                     /gene_synonym="Cln2; LPIC; TPP-1; TPP-I"
                     /inference="alignment:Splign:2.1.0"
     exon            1191..1311
                     /gene="Tpp1"
                     /gene_synonym="Cln2; LPIC; TPP-1; TPP-I"
                     /inference="alignment:Splign:2.1.0"
     exon            1312..1470
                     /gene="Tpp1"
                     /gene_synonym="Cln2; LPIC; TPP-1; TPP-I"
                     /inference="alignment:Splign:2.1.0"
     exon            1471..1596
                     /gene="Tpp1"
                     /gene_synonym="Cln2; LPIC; TPP-1; TPP-I"
                     /inference="alignment:Splign:2.1.0"
     exon            1597..3558
                     /gene="Tpp1"
                     /gene_synonym="Cln2; LPIC; TPP-1; TPP-I"
                     /inference="alignment:Splign:2.1.0"
     regulatory      2428..2433
                     /regulatory_class="polyA_signal_sequence"
                     /gene="Tpp1"
                     /gene_synonym="Cln2; LPIC; TPP-1; TPP-I"
                     /note="hexamer: ATTACA"
     polyA_site      2447
                     /gene="Tpp1"
                     /gene_synonym="Cln2; LPIC; TPP-1; TPP-I"
                     /note="major polyA site"
     regulatory      3531..3536
                     /regulatory_class="polyA_signal_sequence"
                     /gene="Tpp1"
                     /gene_synonym="Cln2; LPIC; TPP-1; TPP-I"
                     /note="hexamer: ATTAAA"
     polyA_site      3540
                     /gene="Tpp1"
                     /gene_synonym="Cln2; LPIC; TPP-1; TPP-I"
ORIGIN      
        1 atgctcatgt gacttatcac atgactacag atcagcctga aagccaaaat gggactccaa
       61 gcccgcctcc tagggctcct tgctctcgtc atcgccggca aatgcactta caaccctgag
      121 ccggaccagc ggtggatgct gcctccaggc tgggtgtccc tgggccgcgt ggatcccgag
      181 gaagagctga gtctcacttt tgcgctgaaa cagcggaacc tggaaagact ctcggagctg
      241 gtgcaggctg tgtcggatcc tagctctcct caatatggaa agtacctaac cctggaggat
      301 gtagctgagc tggttcaacc atcacccctg accctcctca ctgtccaaaa gtggctctca
      361 gcagctggag cccggaactg cgattcagtg accacccagg actttctgac ttgctggctg
      421 agtgtccgac aggctgagct gctgctccca ggagctgagt ttcatcgcta tgtaggggga
      481 cctacaaaga cccatgttat aaggtcccca catccctacc agcttcccca ggccttggcc
      541 cctcatgtgg attttgtggg ggggctgcac cgtttccccc cttcatctcc aagacaacgt
      601 ccagaaccac aacaggtagg aactgttagc ctgcacttgg gagtgactcc gtctgtgctc
      661 cgtcagcgat acaacctgac agccaaagat gtgggctcag gcaccaccaa caatagccag
      721 gcctgtgccc agttcctgga acagtacttc cataactcgg atctgactga gttcatgcgc
      781 ctattcggtg gcagttttac acaccaggcc tcagtagcaa aagttgttgg aaagcaaggg
      841 cgaggccgag ctgggatcga ggccagtcta gatgtggaat acctgatgag tgctggtgcc
      901 aatatctcca cttgggtcta cagtagccct ggccgccatg aggcacagga gcccttctta
      961 caatggctcc tgcttcttag caatgagtca tctttgccac atgtacatac tgtgagttac
     1021 ggagacgatg aagactccct cagcagcatc tacatccaga gagtcaacac tgagttcatg
     1081 aaggctgctg ctcggggtct caccctcctt tttgcctcag gtgacactgg agctgggtgt
     1141 tggtctgtct ccggaagaca caagttccgc cctagcttcc ctgcttccag cccctatgtt
     1201 actacagttg gaggaacctc cttcaagaat cctttcctca tcacagatga agtagttgac
     1261 tatatcagtg gtggaggctt cagcaatgtt ttcccacggc ctccctacca ggaggaagca
     1321 gtggcccagt tcttgaaatc cagctctcat ctaccaccat ccagttactt caatgctagt
     1381 ggccgtgcct acccagatgt tgccgcacta tctgatggct actgggtggt cagcaacatg
     1441 gtccccattc catgggtatc tggaacctcg gcctctactc cagtgtttgg gggaatttta
     1501 tccttgataa atgagcacag aatcctcaat ggccgccctc ctcttggctt tctcaacccc
     1561 aggctctatc agcagcatgg gacaggactc tttgatgtaa cccacggctg ccatgagtcc
     1621 tgtctgaatg aagaagtgga gggtcagggt ttctgctctg gtcctggctg ggatcctgtg
     1681 acaggttggg gaacacccaa cttcccagcc ctactgaaga ccctgctcaa cccttgaccc
     1741 tttcgtgcca tgacgagaaa gcagaactgt tccctgtact aaaagggaag gctcagtttc
     1801 ttgttattcc tcgatagaag ccctgctgaa ctcctgttgc ctgctgcaga tagcttctcc
     1861 ctaaccctca gatgctgtga acaggactca actctcaatc ctactgtgtg ccatcaaact
     1921 caggtctcca aacttctact tcaagatcct caacaagatg ctataaccag catattttgt
     1981 ctcaccccaa ccccatctct ccttcctctt tccagcttga gatgtgaaag cagggcaaga
     2041 aggttcagtc ttccattact gacactagca ggtccaccca acgcttacca cctctgcact
     2101 gaccgtacac tctatttctc ttcgggtttg cttttccgtt cactgaagtg agacctttga
     2161 ctaatcgttt tgtctttctt ctctcggcac tgaagtacaa tggtctcccc aatgttttat
     2221 ccagttatac ccttttcagt gtttgtttta tgggttttct tatttaagaa caggttgtca
     2281 aaaaaccatt aaaaaaaaaa aagaaaaaga acacattggc tgcaatctat ttaactatat
     2341 aactattcta aggaaagtta aaaattgaaa acttaaaatg tttgaaatgt tctcattggc
     2401 aaaattcctc aacaaaataa ataggtcatt acaaattttg ctttaaattt ttgcttgagt
     2461 gatttttttt tttgtaaagt gttttaaaat ttacatttta ttttcccctt ctcagctaca
     2521 ccaaagcaac attcaggttt taatttcaac tgtcagacat tacaaacatc tagcttctgt
     2581 gaacctgggt gtttgttctt tccatcagtt tcccatttat cctttttcct cagtgacccc
     2641 cctctgacca cactaatcag cccccttttc tgctgactcc agggatgctg tgttcattgt
     2701 tccattctta gtttcttgct cttatcattt tattatcatc ccttgacaga tcactgacat
     2761 ctatacccac agtgagtgat acctcaaatg tgagatacct cgttacttca tctccctcca
     2821 gcccagacct aaactatttc atctcttaat ctatgatata atgcctcttt caaacaagcc
     2881 agaaacctat aactcttaac tctcatcttt ttcacctcct tactaacttc aaaaagtttt
     2941 cttgactgcc tctcaagtat atctttatcg gctagtgtgg tgatgcacac ctttaatccc
     3001 agcactcagg agacagaggc caggcaagtc tctgagtttg aggccatcct ggtctatata
     3061 aagagttcca gacaagccag gcctacatag tcagaccatg tctcaaaaat acacatatgt
     3121 gcacacacac acatgcacaa aatactgcat tatctttgag cccatgttct tttttcttct
     3181 aaactgcagg aagcacttgg ggagaggaaa ggcttccgaa gcccctcatc ttccaaacca
     3241 agcagtctat gtatttgtgc aaacccttca aagattactg ggtaaaagtc agagacattg
     3301 aaacttgcct tcaaaatcgg gaaataaaca ttgctaatgt ctcacacttg gaattaagca
     3361 atgaatgtta gtttcccttt cttctttgca ctgcacctac atgtagctgg gaaacaatca
     3421 tacagtattg aaattgtcag tttgtttgcc ttccttttcc agacagtcgg tgctagtgaa
     3481 ctagaatctg attcttaact ttgtattcct aggtcccagc ccaaatagaa attaaataaa
     3541 tgactgttta aaaaaaaaaa aaaaaaaaaa aaa
//
