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Protein

Mitochondrial import inner membrane translocase subunit Tim13

Gene

TIMM13

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Mitochondrial intermembrane chaperone that participates in the import and insertion of some multi-pass transmembrane proteins into the mitochondrial inner membrane. Also required for the transfer of beta-barrel precursors from the TOM complex to the sorting and assembly machinery (SAM complex) of the outer membrane. Acts as a chaperone-like protein that protects the hydrophobic precursors from aggregation and guide them through the mitochondrial intermembrane space. The TIMM8-TIMM13 complex mediates the import of proteins such as TIMM23, SLC25A12/ARALAR1 and SLC25A13/ARALAR2, while the predominant TIMM9-TIMM10 70 kDa complex mediates the import of much more proteins.2 Publications

GO - Molecular functioni

  • protein transporter activity Source: GO_Central
  • zinc ion binding Source: ProtInc

GO - Biological processi

  • chaperone-mediated protein transport Source: BHF-UCL
  • protein import into mitochondrial inner membrane Source: GO_Central
  • protein targeting to mitochondrion Source: ProtInc
  • sensory perception of sound Source: ProtInc

Keywordsi

Molecular functionChaperone
Biological processProtein transport, Translocation, Transport
LigandMetal-binding, Zinc

Enzyme and pathway databases

ReactomeiR-HSA-1268020. Mitochondrial protein import.

Names & Taxonomyi

Protein namesi
Recommended name:
Mitochondrial import inner membrane translocase subunit Tim13
Gene namesi
Name:TIMM13
Synonyms:TIM13B, TIMM13A, TIMM13B
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 19

Organism-specific databases

EuPathDBiHostDB:ENSG00000099800.7.
HGNCiHGNC:11816. TIMM13.

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

Organism-specific databases

OpenTargetsiENSG00000099800.
PharmGKBiPA36522.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00001936231 – 95Mitochondrial import inner membrane translocase subunit Tim13Add BLAST95

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei1N-acetylmethionineCombined sources1
Modified residuei7PhosphoserineCombined sources1
Disulfide bondi46 ↔ 69By similarity
Disulfide bondi50 ↔ 65By similarity
Modified residuei53N6-succinyllysineBy similarity1

Keywords - PTMi

Acetylation, Disulfide bond, Phosphoprotein

Proteomic databases

EPDiQ9Y5L4.
MaxQBiQ9Y5L4.
PaxDbiQ9Y5L4.
PeptideAtlasiQ9Y5L4.
PRIDEiQ9Y5L4.
TopDownProteomicsiQ9Y5L4.

PTM databases

iPTMnetiQ9Y5L4.
PhosphoSitePlusiQ9Y5L4.

Expressioni

Tissue specificityi

Ubiquitous, with highest expression in heart, kidney, liver and skeletal muscle.

Gene expression databases

BgeeiENSG00000099800.
CleanExiHS_TIMM13.
ExpressionAtlasiQ9Y5L4. baseline and differential.
GenevisibleiQ9Y5L4. HS.

Organism-specific databases

HPAiHPA048985.

Interactioni

Subunit structurei

Heterohexamer; composed of 3 copies of TIMM8 (TIMM8A or TIMM8B) and 3 copies of TIMM13, named soluble 70 kDa complex. Associates with the TIM22 complex, whose core is composed of TIMM22.

Protein-protein interaction databases

BioGridi117722. 71 interactors.
CORUMiQ9Y5L4.
IntActiQ9Y5L4. 53 interactors.
MINTiMINT-3087260.
STRINGi9606.ENSP00000215570.

Structurei

3D structure databases

ProteinModelPortaliQ9Y5L4.
SMRiQ9Y5L4.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Motif

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Motifi46 – 69Twin CX3C motifAdd BLAST24

Domaini

The twin CX3C motif contains 4 conserved Cys residues that form 2 disulfide bonds in the mitochondrial intermembrane space. However, during the transit of TIMM13 from cytoplasm into mitochondrion, the Cys residues probably coordinate zinc, thereby preventing folding and allowing its transfer across mitochondrial outer membrane (By similarity).By similarity

Sequence similaritiesi

Belongs to the small Tim family.Curated

Phylogenomic databases

eggNOGiKOG1733. Eukaryota.
ENOG41124YI. LUCA.
GeneTreeiENSGT00390000014000.
HOGENOMiHOG000115759.
HOVERGENiHBG079645.
InParanoidiQ9Y5L4.
KOiK17781.
OMAiDRYMDSW.
OrthoDBiEOG091G138S.
PhylomeDBiQ9Y5L4.
TreeFamiTF106194.

Family and domain databases

Gene3Di1.10.287.810. 1 hit.
InterProiView protein in InterPro
IPR004217. Tim10-like.
IPR035427. Tim10/FAM136A-like_domain.
PfamiView protein in Pfam
PF02953. zf-Tim10_DDP. 1 hit.
SUPFAMiSSF144122. SSF144122. 1 hit.

Sequencei

Sequence statusi: Complete.

Q9Y5L4-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MEGGFGSDFG GSGSGKLDPG LIMEQVKVQI AVANAQELLQ RMTDKCFRKC
60 70 80 90
IGKPGGSLDN SEQKCIAMCM DRYMDAWNTV SRAYNSRLQR ERANM
Length:95
Mass (Da):10,500
Last modified:November 1, 1999 - v1
Checksum:iE40E742C7CA55834
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti2 – 3EG → DS in AAF15101 (PubMed:10552927).Curated2
Sequence conflicti12 – 14SGS → TGG in AAF15101 (PubMed:10552927).Curated3
Sequence conflicti21L → A in AAF15101 (PubMed:10552927).Curated1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF144700 mRNA. Translation: AAD39951.1.
AF152351 mRNA. Translation: AAF15101.1.
AF152352 mRNA. Translation: AAF15102.1.
BC008607 mRNA. Translation: AAH08607.1.
CCDSiCCDS12089.1.
RefSeqiNP_036590.1. NM_012458.3.
UniGeneiHs.75056.

Genome annotation databases

EnsembliENST00000215570; ENSP00000215570; ENSG00000099800.
GeneIDi26517.
KEGGihsa:26517.
UCSCiuc002lvx.3. human.

Similar proteinsi

Entry informationi

Entry nameiTIM13_HUMAN
AccessioniPrimary (citable) accession number: Q9Y5L4
Secondary accession number(s): P62206, Q9UHL8, Q9WTL1
Entry historyiIntegrated into UniProtKB/Swiss-Prot: January 11, 2001
Last sequence update: November 1, 1999
Last modified: September 27, 2017
This is version 149 of the entry and version 1 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. Human chromosome 19
    Human chromosome 19: entries, gene names and cross-references to MIM
  2. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  3. SIMILARITY comments
    Index of protein domains and families