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Protein

DnaJ homolog subfamily A member 3, mitochondrial

Gene

DNAJA3

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

Functioni

Modulates apoptotic signal transduction or effector structures within the mitochondrial matrix. Affect cytochrome C release from the mitochondria and caspase 3 activation, but not caspase 8 activation. Isoform 1 increases apoptosis triggered by both TNF and the DNA-damaging agent mytomycin C; in sharp contrast, isoform 2 suppresses apoptosis. Can modulate IFN-gamma-mediated transcriptional activity. Isoform 2 may play a role in neuromuscular junction development as an effector of the MUSK signaling pathway.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Metal bindingi236Zinc 1Combined sources1 Publication1
Metal bindingi239Zinc 1Combined sources1 Publication1
Metal bindingi253Zinc 2Combined sources1 Publication1
Metal bindingi256Zinc 2Combined sources1 Publication1
Metal bindingi275Zinc 2Combined sources1 Publication1
Metal bindingi278Zinc 2Combined sources1 Publication1
Metal bindingi289Zinc 1Combined sources1 Publication1
Metal bindingi292Zinc 1Combined sources1 Publication1

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Zinc fingeri223 – 301CR-typeAdd BLAST79

GO - Molecular functioni

  • ATP binding Source: InterPro
  • GTPase regulator activity Source: Ensembl
  • heat shock protein binding Source: InterPro
  • interferon-gamma receptor binding Source: UniProtKB
  • metal ion binding Source: UniProtKB-KW
  • NF-kappaB binding Source: UniProtKB
  • protein kinase binding Source: UniProtKB
  • transcription factor binding Source: UniProtKB
  • unfolded protein binding Source: Ensembl

GO - Biological processi

  • activation-induced cell death of T cells Source: Ensembl
  • activation of cysteine-type endopeptidase activity involved in apoptotic process Source: UniProtKB
  • cell aging Source: Ensembl
  • mitochondrial DNA replication Source: Ensembl
  • mitochondrion organization Source: GO_Central
  • negative regulation of apoptotic process Source: UniProtKB
  • negative regulation of cell proliferation Source: UniProtKB
  • negative regulation of cysteine-type endopeptidase activity involved in apoptotic process Source: UniProtKB
  • negative regulation of I-kappaB kinase/NF-kappaB signaling Source: UniProtKB
  • negative regulation of interferon-gamma-mediated signaling pathway Source: UniProtKB
  • negative regulation of NF-kappaB transcription factor activity Source: UniProtKB
  • negative regulation of programmed cell death Source: Ensembl
  • negative regulation of protein kinase activity Source: UniProtKB
  • negative regulation of transcription by RNA polymerase II Source: UniProtKB
  • neuromuscular junction development Source: UniProtKB
  • positive regulation of apoptotic process Source: UniProtKB
  • positive regulation of protein ubiquitination Source: UniProtKB
  • positive regulation of T cell proliferation Source: Ensembl
  • protein folding Source: UniProtKB
  • protein stabilization Source: UniProtKB
  • response to heat Source: InterPro
  • response to interferon-gamma Source: UniProtKB
  • skeletal muscle acetylcholine-gated channel clustering Source: UniProtKB
  • small GTPase mediated signal transduction Source: Ensembl
  • T cell differentiation in thymus Source: Ensembl

Keywordsi

Molecular functionChaperone
Biological processApoptosis
LigandMetal-binding, Zinc

Names & Taxonomyi

Protein namesi
Recommended name:
DnaJ homolog subfamily A member 3, mitochondrial
Alternative name(s):
DnaJ protein Tid-1
Short name:
hTid-1
Hepatocellular carcinoma-associated antigen 57
Tumorous imaginal discs protein Tid56 homolog
Gene namesi
Name:DNAJA3
Synonyms:HCA57, TID1
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi

Organism-specific databases

EuPathDBiHostDB:ENSG00000103423.13
HGNCiHGNC:11808 DNAJA3
MIMi608382 gene
neXtProtiNX_Q96EY1

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

Cell junction, Cell membrane, Cytoplasm, Membrane, Mitochondrion, Postsynaptic cell membrane, Synapse

Pathology & Biotechi

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Mutagenesisi121H → Q: Loss of modulation of apoptosis. 1 Publication1

Organism-specific databases

DisGeNETi9093
OpenTargetsiENSG00000103423
PharmGKBiPA27410

Polymorphism and mutation databases

BioMutaiDNAJA3
DMDMi311033374

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_0000007256? – 480DnaJ homolog subfamily A member 3, mitochondrial
Transit peptidei1 – ?MitochondrionSequence analysis

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei58Omega-N-methylarginine; by CARM11 Publication1
Modified residuei134N6-acetyllysineBy similarity1
Modified residuei238Omega-N-methylarginine; by CARM11 Publication1
Modified residuei293Omega-N-methylarginine; by CARM11 Publication1
Modified residuei398PhosphoserineCombined sources1

Post-translational modificationi

Tyrosine phosphorylated.By similarity

Keywords - PTMi

Acetylation, Methylation, Phosphoprotein

Proteomic databases

EPDiQ96EY1
MaxQBiQ96EY1
PaxDbiQ96EY1
PeptideAtlasiQ96EY1
PRIDEiQ96EY1

PTM databases

iPTMnetiQ96EY1
PhosphoSitePlusiQ96EY1

Expressioni

Tissue specificityi

Widely expressed with highest levels in heart, liver, lung and skeletal muscles. Also expressed in keratinocytes.1 Publication

Gene expression databases

BgeeiENSG00000103423
CleanExiHS_DNAJA3
ExpressionAtlasiQ96EY1 baseline and differential
GenevisibleiQ96EY1 HS

Organism-specific databases

HPAiCAB016095
HPA040875
HPA044229

Interactioni

Subunit structurei

Interacts with JAK2, HSPA9B and IFN-gammaR2 chain. Interacts with Ras GTPase-activating protein 1 (RASA1). Isoform 2 interacts with MUSK (via the cytoplasmic domain) (By similarity).By similarity

Binary interactionsi

Show more details

GO - Molecular functioni

  • heat shock protein binding Source: InterPro
  • interferon-gamma receptor binding Source: UniProtKB
  • NF-kappaB binding Source: UniProtKB
  • protein kinase binding Source: UniProtKB
  • transcription factor binding Source: UniProtKB
  • unfolded protein binding Source: Ensembl

Protein-protein interaction databases

BioGridi114547, 88 interactors
DIPiDIP-33870N
IntActiQ96EY1, 69 interactors
MINTiQ96EY1
STRINGi9606.ENSP00000262375

Structurei

Secondary structure

1480
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Helixi94 – 98Combined sources5
Helixi106 – 119Combined sources14
Turni122 – 124Combined sources3
Helixi131 – 146Combined sources16
Helixi148 – 156Combined sources9
Beta strandi222 – 224Combined sources3
Beta strandi237 – 244Combined sources8
Beta strandi254 – 259Combined sources6
Beta strandi261 – 266Combined sources6
Beta strandi269 – 274Combined sources6
Beta strandi276 – 284Combined sources9
Beta strandi290 – 294Combined sources5
Beta strandi296 – 298Combined sources3

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2CTTNMR-A213-303[»]
2DN9NMR-A93-158[»]
ProteinModelPortaliQ96EY1
SMRiQ96EY1
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiQ96EY1

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini93 – 158JAdd BLAST66
Repeati236 – 243CXXCXGXG motif8
Repeati253 – 260CXXCXGXG motif8
Repeati275 – 282CXXCXGXG motif8
Repeati289 – 296CXXCXGXG motif8

Domaini

Modulation of apoptosis, i.e. proapoptotic activity of isoform 1 and antiapoptotic activity of isoform 2, is J domain-dependent.

Zinc finger

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Zinc fingeri223 – 301CR-typeAdd BLAST79

Keywords - Domaini

Repeat, Transit peptide, Zinc-finger

Phylogenomic databases

eggNOGiKOG0715 Eukaryota
COG0484 LUCA
GeneTreeiENSGT00890000139473
HOGENOMiHOG000203530
HOVERGENiHBG051371
InParanoidiQ96EY1
KOiK09504
OMAiDMGGFAD
OrthoDBiEOG091G09C7
PhylomeDBiQ96EY1
TreeFamiTF105152

Family and domain databases

CDDicd06257 DnaJ, 1 hit
cd10719 DnaJ_zf, 1 hit
Gene3Di1.10.287.110, 1 hit
HAMAPiMF_01152 DnaJ, 1 hit
InterProiView protein in InterPro
IPR012724 DnaJ
IPR002939 DnaJ_C
IPR001623 DnaJ_domain
IPR018253 DnaJ_domain_CS
IPR008971 HSP40/DnaJ_pept-bd
IPR001305 HSP_DnaJ_Cys-rich_dom
IPR036410 HSP_DnaJ_Cys-rich_dom_sf
IPR036869 J_dom_sf
PfamiView protein in Pfam
PF00226 DnaJ, 1 hit
PF01556 DnaJ_C, 1 hit
PF00684 DnaJ_CXXCXGXG, 1 hit
PRINTSiPR00625 JDOMAIN
SMARTiView protein in SMART
SM00271 DnaJ, 1 hit
SUPFAMiSSF46565 SSF46565, 1 hit
SSF49493 SSF49493, 1 hit
SSF57938 SSF57938, 1 hit
PROSITEiView protein in PROSITE
PS00636 DNAJ_1, 1 hit
PS50076 DNAJ_2, 1 hit
PS51188 ZF_CR, 1 hit

Sequences (3)i

Sequence statusi: Complete.

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

This entry describes 3 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: Q96EY1-1) [UniParc]FASTAAdd to basket
Also known as: Tid-1(L)

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
MAARCSTRWL LVVVGTPRLP AISGRGARPP REGVVGAWLS RKLSVPAFAS
60 70 80 90 100
SLTSCGPRAL LTLRPGVSLT GTKHNPFICT ASFHTSAPLA KEDYYQILGV
110 120 130 140 150
PRNASQKEIK KAYYQLAKKY HPDTNKDDPK AKEKFSQLAE AYEVLSDEVK
160 170 180 190 200
RKQYDAYGSA GFDPGASGSQ HSYWKGGPTV DPEELFRKIF GEFSSSSFGD
210 220 230 240 250
FQTVFDQPQE YFMELTFNQA AKGVNKEFTV NIMDTCERCN GKGNEPGTKV
260 270 280 290 300
QHCHYCGGSG METINTGPFV MRSTCRRCGG RGSIIISPCV VCRGAGQAKQ
310 320 330 340 350
KKRVMIPVPA GVEDGQTVRM PVGKREIFIT FRVQKSPVFR RDGADIHSDL
360 370 380 390 400
FISIAQALLG GTARAQGLYE TINVTIPPGT QTDQKIRMGG KGIPRINSYG
410 420 430 440 450
YGDHYIHIKI RVPKRLTSRQ QSLILSYAED ETDVEGTVNG VTLTSSGGST
460 470 480
MDSSAGSKAR REAGEDEEGF LSKLKKMFTS
Length:480
Mass (Da):52,489
Last modified:November 2, 2010 - v2
Checksum:i5A57B9020992CF59
GO
Isoform 2 (identifier: Q96EY1-2) [UniParc]FASTAAdd to basket
Also known as: Tid-1(S)

The sequence of this isoform differs from the canonical sequence as follows:
     448-453: GSTMDS → KRSTGN
     454-480: Missing.

Show »
Length:453
Mass (Da):49,611
Checksum:iE235B8DC1DB99FBC
GO
Isoform 3 (identifier: Q96EY1-3) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     3-186: ARCSTRWLLV...GPTVDPEELF → EPQAERPRLCVFPDLLRPPSAADIETWCQPY
     448-453: GSTMDS → KRSTGN
     454-480: Missing.

Show »
Length:300
Mass (Da):33,082
Checksum:i36D883E568E8ECC8
GO

Sequence cautioni

The sequence AAF66245 differs from that shown. Reason: Frameshift at positions 408 and 411.Curated
The sequence AAF66245 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.Curated
The sequence AAH12343 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti320M → W in AAF66245 (PubMed:12097419).Curated1
Isoform 3 (identifier: Q96EY1-3)
Sequence conflicti33Y → H in BAG58345 (PubMed:14702039).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_02796575N → Y4 PublicationsCorresponds to variant dbSNP:rs1139653Ensembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_0557283 – 186ARCST…PEELF → EPQAERPRLCVFPDLLRPPS AADIETWCQPY in isoform 3. 1 PublicationAdd BLAST184
Alternative sequenceiVSP_007425448 – 453GSTMDS → KRSTGN in isoform 2 and isoform 3. 4 Publications6
Alternative sequenceiVSP_007426454 – 480Missing in isoform 2 and isoform 3. 4 PublicationsAdd BLAST27

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF061749 mRNA Translation: AAC29066.1
AF411044 mRNA Translation: AAL35323.1
AK295391 mRNA Translation: BAG58345.1
AK314218 mRNA Translation: BAG36892.1
AC012676 Genomic DNA No translation available.
BC007225 mRNA Translation: AAH07225.1
BC011855 mRNA Translation: AAH11855.1
BC012343 mRNA Translation: AAH12343.1 Different initiation.
BC014062 mRNA Translation: AAH14062.1
BC020248 mRNA Translation: AAH20248.1
BC030145 mRNA Translation: AAH30145.1
BC032100 mRNA Translation: AAH32100.1
AF244136 mRNA Translation: AAF66245.1 Sequence problems.
CCDSiCCDS10515.1 [Q96EY1-1]
CCDS45400.1 [Q96EY1-2]
CCDS66930.1 [Q96EY1-3]
RefSeqiNP_001128582.1, NM_001135110.2 [Q96EY1-2]
NP_001273445.1, NM_001286516.1 [Q96EY1-3]
NP_005138.3, NM_005147.5 [Q96EY1-1]
UniGeneiHs.459779

Genome annotation databases

EnsembliENST00000262375; ENSP00000262375; ENSG00000103423 [Q96EY1-1]
ENST00000355296; ENSP00000347445; ENSG00000103423 [Q96EY1-2]
ENST00000431375; ENSP00000393970; ENSG00000103423 [Q96EY1-3]
ENST00000612103; ENSP00000477570; ENSG00000276726 [Q96EY1-1]
ENST00000614397; ENSP00000479815; ENSG00000276726 [Q96EY1-2]
GeneIDi9093
KEGGihsa:9093
UCSCiuc002cwk.4 human [Q96EY1-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Entry informationi

Entry nameiDNJA3_HUMAN
AccessioniPrimary (citable) accession number: Q96EY1
Secondary accession number(s): B2RAJ5
, B4DI33, E7ES32, O75472, Q8WUJ6, Q8WXJ3, Q96D76, Q96IV1, Q9NYH8
Entry historyiIntegrated into UniProtKB/Swiss-Prot: May 16, 2003
Last sequence update: November 2, 2010
Last modified: May 23, 2018
This is version 172 of the entry and version 2 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

3D-structure, Complete proteome, Reference proteome

Documents

  1. Human chromosome 16
    Human chromosome 16: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references

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