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Protein

Mitochondrial import inner membrane translocase subunit TIM14

Gene

DNAJC19

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

Functioni

Probable component of the PAM complex, a complex required for the translocation of transit peptide-containing proteins from the inner membrane into the mitochondrial matrix in an ATP-dependent manner. May act as a co-chaperone that stimulate the ATP-dependent activity (By similarity).By similarity

GO - Biological processi

  • genitalia development Source: UniProtKB
  • protein folding Source: UniProtKB
  • protein targeting to mitochondrion Source: UniProtKB
  • visual perception Source: UniProtKB

Keywordsi

Molecular functionChaperone
Biological processProtein transport, Translocation, Transport

Enzyme and pathway databases

ReactomeiR-HSA-1268020 Mitochondrial protein import

Names & Taxonomyi

Protein namesi
Recommended name:
Mitochondrial import inner membrane translocase subunit TIM14
Alternative name(s):
DnaJ homolog subfamily C member 19
Gene namesi
Name:DNAJC19
Synonyms:TIM14, TIMM14
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 3

Organism-specific databases

EuPathDBiHostDB:ENSG00000205981.6
HGNCiHGNC:30528 DNAJC19
MIMi608977 gene
neXtProtiNX_Q96DA6

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

Topology

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Topological domaini2 – 3Mitochondrial intermembraneSequence analysis2
Transmembranei4 – 24HelicalSequence analysisAdd BLAST21
Topological domaini25 – 116Mitochondrial matrixSequence analysisAdd BLAST92

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion inner membrane

Pathology & Biotechi

Involvement in diseasei

3-methylglutaconic aciduria 5 (MGA5)1 Publication
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionAn autosomal recessive disorder characterized by early-onset dilated cardiomyopathy, growth failure, cerebellar ataxia causing significant motor delays, testicular dysgenesis, growth failure and significant increases in urine organic acids, particularly 3-methylglutaconic acid and 3-methylglutaric acid.
See also OMIM:610198

Keywords - Diseasei

Cardiomyopathy

Organism-specific databases

DisGeNETi131118
MalaCardsiDNAJC19
MIMi610198 phenotype
OpenTargetsiENSG00000205981
Orphaneti66634 Dilated cardiomyopathy with ataxia
PharmGKBiPA142671967

Polymorphism and mutation databases

BioMutaiDNAJC19
DMDMi74760780

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Initiator methionineiRemovedCombined sources1 Publication
ChainiPRO_00000711002 – 116Mitochondrial import inner membrane translocase subunit TIM14Add BLAST115

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei2N-acetylalanineCombined sources1 Publication1
Modified residuei39PhosphoserineCombined sources1
Modified residuei70PhosphoserineCombined sources1

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

EPDiQ96DA6
MaxQBiQ96DA6
PaxDbiQ96DA6
PeptideAtlasiQ96DA6
PRIDEiQ96DA6
ProteomicsDBi76265
TopDownProteomicsiQ96DA6-1 [Q96DA6-1]

PTM databases

iPTMnetiQ96DA6
PhosphoSitePlusiQ96DA6

Expressioni

Tissue specificityi

Ubiquitously expressed.

Gene expression databases

BgeeiENSG00000205981
CleanExiHS_DNAJC19
ExpressionAtlasiQ96DA6 baseline and differential
GenevisibleiQ96DA6 HS

Organism-specific databases

HPAiHPA037782

Interactioni

Subunit structurei

Probable component of the PAM complex at least composed of a mitochondrial HSP70 protein, GRPEL1 or GRPEL2, TIMM44, TIMM16/PAM16 and TIMM14/DNAJC19.By similarity

Protein-protein interaction databases

BioGridi126272, 35 interactors
DIPiDIP-62091N
IntActiQ96DA6, 3 interactors
STRINGi9606.ENSP00000372005

Structurei

3D structure databases

ProteinModelPortaliQ96DA6
SMRiQ96DA6
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini62 – 116JPROSITE-ProRule annotationAdd BLAST55

Sequence similaritiesi

Belongs to the TIM14 family.Curated

Keywords - Domaini

Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG0723 Eukaryota
COG2214 LUCA
GeneTreeiENSGT00390000017303
HOGENOMiHOG000115841
HOVERGENiHBG057483
InParanoidiQ96DA6
KOiK09539
OMAiFDAESMA
OrthoDBiEOG091G116P
PhylomeDBiQ96DA6
TreeFamiTF320584

Family and domain databases

CDDicd06257 DnaJ, 1 hit
Gene3Di1.10.287.110, 1 hit
InterProiView protein in InterPro
IPR001623 DnaJ_domain
IPR036869 J_dom_sf
PfamiView protein in Pfam
PF00226 DnaJ, 1 hit
SMARTiView protein in SMART
SM00271 DnaJ, 1 hit
SUPFAMiSSF46565 SSF46565, 1 hit
PROSITEiView protein in PROSITE
PS50076 DNAJ_2, 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: Q96DA6-1) [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
MASTVVAVGL TIAAAGFAGR YVLQAMKHME PQVKQVFQSL PKSAFSGGYY
60 70 80 90 100
RGGFEPKMTK REAALILGVS PTANKGKIRD AHRRIMLLNH PDKGGSPYIA
110
AKINEAKDLL EGQAKK
Length:116
Mass (Da):12,499
Last modified:January 23, 2007 - v3
Checksum:iFEEFD5D2AE5D15F2
GO
Isoform 2 (identifier: Q96DA6-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-25: Missing.

Note: Gene prediction based on EST data.
Show »
Length:91
Mass (Da):10,079
Checksum:i21845A478B7A9F97
GO

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_0471191 – 25Missing in isoform 2. CuratedAdd BLAST25

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AK311765 mRNA Translation: BAG34708.1
AC008009 Genomic DNA No translation available.
CH471052 Genomic DNA Translation: EAW78358.1
CH471052 Genomic DNA Translation: EAW78360.1
CH471052 Genomic DNA Translation: EAW78362.1
BC073989 mRNA Translation: AAH73989.1
BC009702 mRNA Translation: AAH09702.1
CCDSiCCDS33895.1 [Q96DA6-1]
CCDS54684.1 [Q96DA6-2]
RefSeqiNP_001177162.1, NM_001190233.1 [Q96DA6-2]
NP_660304.1, NM_145261.3 [Q96DA6-1]
UniGeneiHs.230601

Genome annotation databases

EnsembliENST00000382564; ENSP00000372005; ENSG00000205981 [Q96DA6-1]
ENST00000479269; ENSP00000419191; ENSG00000205981 [Q96DA6-2]
ENST00000491873; ENSP00000420767; ENSG00000205981 [Q96DA6-2]
GeneIDi131118
KEGGihsa:131118
UCSCiuc003fkt.4 human [Q96DA6-1]

Keywords - Coding sequence diversityi

Alternative splicing

Similar proteinsi

Entry informationi

Entry nameiTIM14_HUMAN
AccessioniPrimary (citable) accession number: Q96DA6
Secondary accession number(s): B2R4B1, C9JBV1
Entry historyiIntegrated into UniProtKB/Swiss-Prot: December 6, 2005
Last sequence update: January 23, 2007
Last modified: June 20, 2018
This is version 138 of the entry and version 3 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, Direct protein sequencing, Reference proteome

Documents

  1. Human chromosome 3
    Human chromosome 3: 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

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