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Protein

Tetratricopeptide repeat protein 19, mitochondrial

Gene

Ttc19

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

Required for the preservation of the structural and functional integrity of mitochondrial respiratory complex III by allowing the physiological turnover of the Rieske protein UQCRFS1 (PubMed:21278747, PubMed:28673544). Involved in the clearance of UQCRFS1 N-terminal fragments, which are produced upon incorporation into the complex III and whose presence is detrimental for its catalytic activity (PubMed:28673544).2 Publications

GO - Biological processi

Keywordsi

Biological processElectron transport, Respiratory chain, Transport

Names & Taxonomyi

Protein namesi
Recommended name:
Tetratricopeptide repeat protein 19, mitochondrial
Short name:
TPR repeat protein 19
Gene namesi
Name:Ttc19
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 11

Organism-specific databases

MGIiMGI:1920045 Ttc19

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion inner membrane

Pathology & Biotechi

Disruption phenotypei

Mutant mice show progressive neurological and energy metabolic decline, decrease complex III activity, increased production of reactive oxygen species, extensive astrogliosis and accumulation of ubiquitinated proteins in neurons of the thalamus. Compatible with a Mendelian autosomal recessive trait. No evidence of embryonic lethality nor evidence of obvious neuronal loss.1 Publication

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 52MitochondrionSequence analysisAdd BLAST52
ChainiPRO_000010640853 – 365Tetratricopeptide repeat protein 19, mitochondrialAdd BLAST313

Proteomic databases

PaxDbiQ8CC21
PeptideAtlasiQ8CC21
PRIDEiQ8CC21

PTM databases

iPTMnetiQ8CC21
PhosphoSitePlusiQ8CC21

Expressioni

Gene expression databases

BgeeiENSMUSG00000042298
CleanExiMM_TTC19
ExpressionAtlasiQ8CC21 baseline and differential
GenevisibleiQ8CC21 MM

Interactioni

Subunit structurei

Binds to the mature mitochondrial complex III dimer, after the incorporation of the Rieske protein UQCRFS1 (PubMed:28673544, PubMed:21278747). Interacts with UQCRC1 and UQCRFS1 (PubMed:21278747). Interacts with ZFYVE26 and CHMP4B (By similarity).By similarity2 Publications

Protein-protein interaction databases

STRINGi10090.ENSMUSP00000054367

Structurei

3D structure databases

ProteinModelPortaliQ8CC21
SMRiQ8CC21
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Repeati127 – 160TPR 1Add BLAST34
Repeati220 – 260TPR 2Add BLAST41
Repeati269 – 302TPR 3Add BLAST34
Repeati308 – 341TPR 4Add BLAST34

Sequence similaritiesi

Belongs to the TTC19 family.Curated

Keywords - Domaini

Repeat, TPR repeat, Transit peptide

Phylogenomic databases

eggNOGiKOG1840 Eukaryota
COG0457 LUCA
GeneTreeiENSGT00390000009194
HOGENOMiHOG000015406
HOVERGENiHBG056672
InParanoidiQ8CC21
KOiK18169
OMAiGMKQEDN
OrthoDBiEOG091G0AH0
PhylomeDBiQ8CC21
TreeFamiTF314010

Family and domain databases

Gene3Di1.25.40.10, 2 hits
InterProiView protein in InterPro
IPR013026 TPR-contain_dom
IPR011990 TPR-like_helical_dom_sf
IPR019734 TPR_repeat
SMARTiView protein in SMART
SM00028 TPR, 4 hits
SUPFAMiSSF48452 SSF48452, 1 hit
PROSITEiView protein in PROSITE
PS50293 TPR_REGION, 1 hit

Sequences (2)i

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: Q8CC21-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide

        10         20         30         40         50
MFRLLRWRLG RTLLRAAGRR CGGCTARLLP ERTGDAGTGA ERLRTRGAPA
60 70 80 90 100
RGHGVLPLLA ALAWFSRPAA TAEQPGEDAS DEAEAEIIQL LKQAKLSIMK
110 120 130 140 150
DEPEAAELIL HDALRLAYES DNRKAITYTY DLMANLAFIR GQLENAEQLF
160 170 180 190 200
KATMSYLLGG GMKQEDNAII EISLKLANIY AAQNKQEFAL AGYEFCISTL
210 220 230 240 250
EGKIEREKEL AEDIMSEETA NTYLLLGMCL DSCARYLLFS KQLSQAQRMY
260 270 280 290 300
EKALQICQEI QGERHPQTIV LMSDLATTLD AQGHFDDAYI YMQRASDLAR
310 320 330 340 350
EINHPELHMV LSNLAAILIH RERYTQAKEI YQEALKRAEL KRDEVSVQHI
360
REELAELSRK SRRLT
Length:365
Mass (Da):41,234
Last modified:March 1, 2003 - v1
Checksum:i2C1AD6DCD91C3B69
GO
Isoform 2 (identifier: Q8CC21-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     133-145: Missing.

Show »
Length:352
Mass (Da):39,776
Checksum:iD8E433AFFE28BB33
GO

Sequence cautioni

The sequence AAH37111 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.Curated
The sequence BAB28813 differs from that shown. Reason: Frameshift at positions 21 and 42.Curated
The sequence EDL10340 differs from that shown. Reason: Erroneous gene model prediction.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti43L → V in BAB28813 (PubMed:16141072).Curated1
Sequence conflicti220A → S in BAB28813 (PubMed:16141072).Curated1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_041051133 – 145Missing in isoform 2. 1 PublicationAdd BLAST13

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AK013368 mRNA Translation: BAB28813.2 Frameshift.
AK034075 mRNA Translation: BAC28572.1
AL596110 Genomic DNA Translation: CAI35326.1
AL596110 Genomic DNA Translation: CAI35327.1
CH466601 Genomic DNA Translation: EDL10340.1 Sequence problems.
BC026866 mRNA Translation: AAH26866.1
BC037111 mRNA Translation: AAH37111.1 Different initiation.
CCDSiCCDS36177.1 [Q8CC21-1]
CCDS48817.1 [Q8CC21-2]
RefSeqiNP_082636.3, NM_028360.2 [Q8CC21-1]
NP_083980.2, NM_029704.2 [Q8CC21-2]
UniGeneiMm.489722

Genome annotation databases

EnsembliENSMUST00000050646; ENSMUSP00000054367; ENSMUSG00000042298 [Q8CC21-1]
ENSMUST00000101075; ENSMUSP00000098636; ENSMUSG00000042298 [Q8CC21-2]
GeneIDi72795
KEGGimmu:72795
UCSCiuc007jir.2 mouse [Q8CC21-1]
uc007jis.2 mouse [Q8CC21-2]

Keywords - Coding sequence diversityi

Alternative splicing

Similar proteinsi

Entry informationi

Entry nameiTTC19_MOUSE
AccessioniPrimary (citable) accession number: Q8CC21
Secondary accession number(s): Q5NCZ3
, Q5NCZ4, Q8K1X2, Q8R0G6, Q9CYS5
Entry historyiIntegrated into UniProtKB/Swiss-Prot: September 27, 2004
Last sequence update: March 1, 2003
Last modified: May 23, 2018
This is version 118 of the entry and version 1 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

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