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Protein

Mitochondrial import inner membrane translocase subunit TIM14

Gene

DNAJC19

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-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

HGNCiHGNC:30528. DNAJC19.

Subcellular locationi

Topology

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

GO - Cellular componenti

  • integral component of membrane Source: UniProtKB
  • mitochondrial inner membrane Source: GO_Central
  • mitochondrion Source: UniProtKB
  • protein complex Source: GO_Central

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.
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. 34 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.
OMAiKDFMDNA.
OrthoDBiEOG091G116P.
PhylomeDBiQ96DA6.
TreeFamiTF320584.

Family and domain databases

CDDicd06257. DnaJ. 1 hit.
Gene3Di1.10.287.110. 1 hit.
InterProiView protein in InterPro
IPR001623. DnaJ_domain.
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: August 30, 2017
This is version 132 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