LOCUS       NP_082636                365 aa            linear   ROD 05-MAY-2025
DEFINITION  tetratricopeptide repeat protein 19, mitochondrial isoform 1 [Mus
            musculus].
ACCESSION   NP_082636
VERSION     NP_082636.3
DBSOURCE    REFSEQ: accession NM_028360.3
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  (residues 1 to 365)
  AUTHORS   Radaelli,E., Assenmacher,C.A., Verrelle,J., Banerjee,E., Manero,F.,
            Khiati,S., Girona,A., Lopez-Lluch,G., Navas,P. and Spinazzi,M.
  TITLE     Mitochondrial defects caused by PARL deficiency lead to arrested
            spermatogenesis and ferroptosis
  JOURNAL   Elife 12, e84710 (2023)
   PUBMED   37505079
  REMARK    Erratum:[Elife. 2024 Sep 13;13:e103278. doi: 10.7554/eLife.103278.
            PMID: 39268597]
            Publication Status: Online-Only
REFERENCE   2  (residues 1 to 365)
  AUTHORS   Spinazzi,M., Radaelli,E., Horre,K., Arranz,A.M., Gounko,N.V.,
            Agostinis,P., Maia,T.M., Impens,F., Morais,V.A., Lopez-Lluch,G.,
            Serneels,L., Navas,P. and De Strooper,B.
  TITLE     PARL deficiency in mouse causes Complex III defects, coenzyme Q
            depletion, and Leigh-like syndrome
  JOURNAL   Proc Natl Acad Sci U S A 116 (1), 277-286 (2019)
   PUBMED   30578322
REFERENCE   3  (residues 1 to 365)
  AUTHORS   Bottani,E., Cerutti,R., Harbour,M.E., Ravaglia,S., Dogan,S.A.,
            Giordano,C., Fearnley,I.M., D'Amati,G., Viscomi,C.,
            Fernandez-Vizarra,E. and Zeviani,M.
  TITLE     TTC19 Plays a Husbandry Role on UQCRFS1 Turnover in the Biogenesis
            of Mitochondrial Respiratory Complex III
  JOURNAL   Mol Cell 67 (1), 96-105 (2017)
   PUBMED   28673544
  REMARK    GeneRIF: UQCRFS1 produces N-terminal polypeptides, which remain
            bound to holocomplex III. We show that, in normal conditions, these
            UQCRFS1 fragments are rapidly removed, but when TTC19 is absent
            they accumulate within complex III, causing its structural and
            functional impairment.
REFERENCE   4  (residues 1 to 365)
  AUTHORS   Ghezzi,D., Arzuffi,P., Zordan,M., Da Re,C., Lamperti,C., Benna,C.,
            D'Adamo,P., Diodato,D., Costa,R., Mariotti,C., Uziel,G.,
            Smiderle,C. and Zeviani,M.
  TITLE     Mutations in TTC19 cause mitochondrial complex III deficiency and
            neurological impairment in humans and flies
  JOURNAL   Nat Genet 43 (3), 259-263 (2011)
   PUBMED   21278747
REFERENCE   5  (residues 1 to 365)
  AUTHORS   Powles,N., Babbs,C., Ficker,M., Schimmang,T. and Maconochie,M.
  TITLE     Identification and analysis of genes from the mouse otic vesicle
            and their association with developmental subprocesses through in
            situ hybridization
  JOURNAL   Dev Biol 268 (1), 24-38 (2004)
   PUBMED   15031102
COMMENT     VALIDATED REFSEQ: This record has undergone validation or
            preliminary review. The reference sequence was derived from
            AL596110.15.
            
            On Jul 14, 2006 this sequence version replaced NP_082636.2.
            
            Transcript Variant: This variant (1) encodes the longest isoform
            (1).
            
            ##Evidence-Data-START##
            Transcript exon combination :: AK034075.1, SRR7974084.3704.1
                                           [ECO:0000332]
            RNAseq introns              :: single sample supports all introns
                                           SAMN00849374, SAMN00849375
                                           [ECO:0000348]
            ##Evidence-Data-END##
            
            ##RefSeq-Attributes-START##
            gene product(s) localized to mito. :: reported by MitoCarta
            RefSeq Select criteria             :: based on conservation,
                                                  expression, longest protein
            ##RefSeq-Attributes-END##
FEATURES             Location/Qualifiers
     source          1..365
                     /organism="Mus musculus"
                     /strain="C57BL/6"
                     /db_xref="taxon:10090"
                     /chromosome="11"
                     /map="11 38.02 cM"
     Protein         1..365
                     /product="tetratricopeptide repeat protein 19,
                     mitochondrial isoform 1"
                     /note="tetratricopeptide repeat protein 19, mitochondrial;
                     TPR repeat protein 19"
                     /calculated_mol_wt=35544
     transit_peptide 1..52
                     /note="Mitochondrion. /evidence=ECO:0000255; propagated
                     from UniProtKB/Swiss-Prot (Q8CC21.1)"
                     /calculated_mol_wt=5709
     mat_peptide     53..365
                     /product="Tetratricopeptide repeat protein 19,
                     mitochondrial. /id=PRO_0000106408"
                     /note="propagated from UniProtKB/Swiss-Prot (Q8CC21.1)"
                     /calculated_mol_wt=35544
     Site            70..71
                     /site_type="cleavage"
                     /note="Cleavage, by PARL.
                     /evidence=ECO:0000250|UniProtKB:Q6DKK2; propagated from
                     UniProtKB/Swiss-Prot (Q8CC21.1)"
     Region          127..160
                     /region_name="TPR 1"
                     /note="propagated from UniProtKB/Swiss-Prot (Q8CC21.1)"
     Region          163..>338
                     /region_name="Spy"
                     /note="Predicted O-linked N-acetylglucosamine transferase,
                     SPINDLY family [Posttranslational modification, protein
                     turnover, chaperones]; COG3914"
                     /db_xref="CDD:443119"
     Region          220..260
                     /region_name="TPR 2"
                     /note="propagated from UniProtKB/Swiss-Prot (Q8CC21.1)"
     Region          220..255
                     /region_name="TPR repeat"
                     /note="TPR repeat [structural motif]"
                     /db_xref="CDD:276809"
     Site            order(221,224..225,228..229,238,270,273..274,277..278,
                     280..281,309,312..313,316..317,320)
                     /site_type="other"
                     /note="putative protein binding surface [polypeptide
                     binding]"
                     /db_xref="CDD:276809"
     Region          268..298
                     /region_name="TPR repeat"
                     /note="TPR repeat [structural motif]"
                     /db_xref="CDD:276809"
     Region          269..302
                     /region_name="TPR 3"
                     /note="propagated from UniProtKB/Swiss-Prot (Q8CC21.1)"
     Region          308..341
                     /region_name="TPR 4"
                     /note="propagated from UniProtKB/Swiss-Prot (Q8CC21.1)"
     Region          308..336
                     /region_name="TPR repeat"
                     /note="TPR repeat [structural motif]"
                     /db_xref="CDD:276809"
     CDS             1..365
                     /gene="Ttc19"
                     /gene_synonym="2010204O13Rik; 2810460C24Rik"
                     /coded_by="NM_028360.3:41..1138"
                     /note="isoform 1 is encoded by transcript variant 1"
                     /db_xref="CCDS:CCDS36177.1"
                     /db_xref="GeneID:72795"
                     /db_xref="MGI:MGI:1920045"
ORIGIN      
        1 mfrllrwrlg rtllraagrr cggctarllp ertgdagtga erlrtrgapa rghgvlplla
       61 alawfsrpaa taeqpgedas deaeaeiiql lkqaklsimk depeaaelil hdalrlayes
      121 dnrkaityty dlmanlafir gqlenaeqlf katmsyllgg gmkqednaii eislklaniy
      181 aaqnkqefal agyefcistl egkierekel aedimseeta ntylllgmcl dscaryllfs
      241 kqlsqaqrmy ekalqicqei qgerhpqtiv lmsdlattld aqghfddayi ymqrasdlar
      301 einhpelhmv lsnlaailih rerytqakei yqealkrael krdevsvqhi reelaelsrk
      361 srrlt
//
