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P25686 (DNJB2_HUMAN) Reviewed, UniProtKB/Swiss-Prot

Last modified April 16, 2014. Version 134. Feed History...

Clusters with 100%, 90%, 50% identity | Documents (6) | Third-party data text xml rdf/xml gff fasta
to top of pageNames·Attributes·General annotation·Ontologies·Alt products·Sequence annotation·Sequences·References·Cross-refs·Entry info·DocumentsCustomize order

Names and origin

Protein namesRecommended name:
DnaJ homolog subfamily B member 2
Alternative name(s):
DnaJ protein homolog 1
Heat shock 40 kDa protein 3
Heat shock protein J1
Short name=HSJ-1
Gene names
Name:DNAJB2
Synonyms:HSJ1, HSPF3
OrganismHomo sapiens (Human) [Reference proteome]
Taxonomic identifier9606 [NCBI]
Taxonomic lineageEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo

Protein attributes

Sequence length324 AA.
Sequence statusComplete.
Sequence processingThe displayed sequence is further processed into a mature form.
Protein existenceEvidence at protein level

General annotation (Comments)

Tissue specificity

Brain (neuronal layers). Weakly, in skeletal muscle and spleen.

Involvement in disease

Distal spinal muscular atrophy, autosomal recessive, 5 (DSMA5) [MIM:614881]: An autosomal recessive neurologic disorder characterized by young adult onset of slowly progressive distal muscle weakness and atrophy resulting in gait impairment and loss of reflexes due to impaired function of motor nerves. Sensation and cognition are not impaired.
Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.7

Sequence similarities

Contains 1 J domain.

Contains 2 UIM (ubiquitin-interacting motif) repeats.

Sequence caution

Isoform 3: The sequence AAA09035.1 differs from that shown. Reason: Frameshift at position 288.

Ontologies

Keywords
   Coding sequence diversityAlternative splicing
Polymorphism
   DiseaseNeurodegeneration
   DomainRepeat
   Molecular functionChaperone
   PTMAcetylation
   Technical term3D-structure
Complete proteome
Reference proteome
Gene Ontology (GO)
   Biological_processER-associated ubiquitin-dependent protein catabolic process

Inferred from direct assay PubMed 15936278. Source: BHF-UCL

cell death

Inferred from electronic annotation. Source: UniProtKB-KW

negative regulation of cell growth

Inferred from genetic interaction PubMed 9553041. Source: UniProtKB

negative regulation of cell proliferation

Inferred from genetic interaction PubMed 9553041. Source: UniProtKB

negative regulation of inclusion body assembly

Inferred from direct assay PubMed 15936278. Source: BHF-UCL

negative regulation of protein deubiquitination

Inferred from direct assay PubMed 15936278. Source: BHF-UCL

positive regulation of proteasomal ubiquitin-dependent protein catabolic process

Inferred from direct assay PubMed 15936278. Source: BHF-UCL

positive regulation of protein ubiquitination

Inferred from direct assay PubMed 15936278. Source: BHF-UCL

protein folding

Traceable author statement Ref.1. Source: ProtInc

response to unfolded protein

Traceable author statement Ref.1. Source: ProtInc

   Cellular_componentinclusion body

Inferred from direct assay PubMed 15936278. Source: BHF-UCL

   Molecular_functionHsp70 protein binding

Inferred from physical interaction PubMed 15936278. Source: BHF-UCL

polyubiquitin binding

Inferred from direct assay PubMed 15936278. Source: BHF-UCL

proteasome binding

Inferred from direct assay PubMed 15936278. Source: BHF-UCL

unfolded protein binding

Traceable author statement Ref.1. Source: ProtInc

Complete GO annotation...

Alternative products

This entry describes 2 isoforms produced by alternative splicing. [Align] [Select]

Note: Experimental confirmation may be lacking for some isoforms.
Isoform 3 (identifier: P25686-3)

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.
Note: May be produced at very low levels due to a premature stop codon in the mRNA, leading to nonsense-mediated mRNA decay.
Isoform 2 (identifier: P25686-2)

Also known as: HSJ1A;

The sequence of this isoform differs from the canonical sequence as follows:
     275-277: GGR → DVF
     278-324: Missing.

Sequence annotation (Features)

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifier

Molecule processing

Initiator methionine11Removed By similarity
Chain2 – 324323DnaJ homolog subfamily B member 2
PRO_0000071018

Regions

Domain2 – 7170J
Repeat210 – 22617UIM 1
Repeat250 – 26920UIM 2

Amino acid modifications

Modified residue21N-acetylalanine By similarity

Natural variations

Alternative sequence275 – 2773GGR → DVF in isoform 2.
VSP_001286
Alternative sequence278 – 32447Missing in isoform 2.
VSP_001287
Natural variant2701G → R.
Corresponds to variant rs34127289 [ dbSNP | Ensembl ].
VAR_048910

Experimental info

Sequence conflict2141L → R in AAA09034. Ref.1
Sequence conflict2141L → R in AAA09035. Ref.1

Secondary structure

............ 324
Helix Strand Turn

Details...

Sequences

Sequence LengthMass (Da)Tools
Isoform 3 [UniParc].

Last modified September 11, 2007. Version 3.
Checksum: 0154ED3E29F34B4A

FASTA32435,580
        10         20         30         40         50         60 
MASYYEILDV PRSASADDIK KAYRRKALQW HPDKNPDNKE FAEKKFKEVA EAYEVLSDKH 

        70         80         90        100        110        120 
KREIYDRYGR EGLTGTGTGP SRAEAGSGGP GFTFTFRSPE EVFREFFGSG DPFAELFDDL 

       130        140        150        160        170        180 
GPFSELQNRG SRHSGPFFTF SSSFPGHSDF SSSSFSFSPG AGAFRSVSTS TTFVQGRRIT 

       190        200        210        220        230        240 
TRRIMENGQE RVEVEEDGQL KSVTINGVPD DLALGLELSR REQQPSVTSR SGGTQVQQTP 

       250        260        270        280        290        300 
ASCPLDSDLS EDEDLQLAMA YSLSEMEAAG KKPAGGREAQ HRRQGRPKAQ HQDPGLGGTQ 

       310        320 
EGARGEATKR SPSPEEKASR CLIL 

« Hide

Isoform 2 (HSJ1A) [UniParc].

Checksum: 1C843287D7856CFB
Show »

FASTA27730,569

References

« Hide 'large scale' references
[1]"Human homologues of the bacterial heat-shock protein DnaJ are preferentially expressed in neurons."
Cheetham M.E., Brion J.-P., Anderton B.H.
Biochem. J. 284:469-476(1992) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA] (ISOFORM 2).
Tissue: Brain.
[2]Cheetham M.E.
Submitted (JUL-1998) to UniProtKB
Cited for: SEQUENCE REVISION TO 214.
[3]"Cloning of human full-length CDSs in BD Creator(TM) system donor vector."
Kalnine N., Chen X., Rolfs A., Halleck A., Hines L., Eisenstein S., Koundinya M., Raphael J., Moreira D., Kelley T., LaBaer J., Lin Y., Phelan M., Farmer A.
Submitted (MAY-2003) to the EMBL/GenBank/DDBJ databases
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 3).
[4]"Complete sequencing and characterization of 21,243 full-length human cDNAs."
Ota T., Suzuki Y., Nishikawa T., Otsuki T., Sugiyama T., Irie R., Wakamatsu A., Hayashi K., Sato H., Nagai K., Kimura K., Makita H., Sekine M., Obayashi M., Nishi T., Shibahara T., Tanaka T., Ishii S. expand/collapse author list , Yamamoto J., Saito K., Kawai Y., Isono Y., Nakamura Y., Nagahari K., Murakami K., Yasuda T., Iwayanagi T., Wagatsuma M., Shiratori A., Sudo H., Hosoiri T., Kaku Y., Kodaira H., Kondo H., Sugawara M., Takahashi M., Kanda K., Yokoi T., Furuya T., Kikkawa E., Omura Y., Abe K., Kamihara K., Katsuta N., Sato K., Tanikawa M., Yamazaki M., Ninomiya K., Ishibashi T., Yamashita H., Murakawa K., Fujimori K., Tanai H., Kimata M., Watanabe M., Hiraoka S., Chiba Y., Ishida S., Ono Y., Takiguchi S., Watanabe S., Yosida M., Hotuta T., Kusano J., Kanehori K., Takahashi-Fujii A., Hara H., Tanase T.-O., Nomura Y., Togiya S., Komai F., Hara R., Takeuchi K., Arita M., Imose N., Musashino K., Yuuki H., Oshima A., Sasaki N., Aotsuka S., Yoshikawa Y., Matsunawa H., Ichihara T., Shiohata N., Sano S., Moriya S., Momiyama H., Satoh N., Takami S., Terashima Y., Suzuki O., Nakagawa S., Senoh A., Mizoguchi H., Goto Y., Shimizu F., Wakebe H., Hishigaki H., Watanabe T., Sugiyama A., Takemoto M., Kawakami B., Yamazaki M., Watanabe K., Kumagai A., Itakura S., Fukuzumi Y., Fujimori Y., Komiyama M., Tashiro H., Tanigami A., Fujiwara T., Ono T., Yamada K., Fujii Y., Ozaki K., Hirao M., Ohmori Y., Kawabata A., Hikiji T., Kobatake N., Inagaki H., Ikema Y., Okamoto S., Okitani R., Kawakami T., Noguchi S., Itoh T., Shigeta K., Senba T., Matsumura K., Nakajima Y., Mizuno T., Morinaga M., Sasaki M., Togashi T., Oyama M., Hata H., Watanabe M., Komatsu T., Mizushima-Sugano J., Satoh T., Shirai Y., Takahashi Y., Nakagawa K., Okumura K., Nagase T., Nomura N., Kikuchi H., Masuho Y., Yamashita R., Nakai K., Yada T., Nakamura Y., Ohara O., Isogai T., Sugano S.
Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 2).
Tissue: Lung.
[5]"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
The MGC Project Team
Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORMS 2 AND 3).
Tissue: Colon and Lymph.
[6]"An unappreciated role for RNA surveillance."
Hillman R.T., Green R.E., Brenner S.E.
Genome Biol. 5:R8.1-R8.16(2004) [PubMed] [Europe PMC] [Abstract]
Cited for: SPLICE ISOFORM(S) THAT ARE POTENTIAL NMD TARGET(S).
[7]"A rare recessive distal hereditary motor neuropathy with HSJ1 chaperone mutation."
Blumen S.C., Astord S., Robin V., Vignaud L., Toumi N., Cieslik A., Achiron A., Carasso R.L., Gurevich M., Braverman I., Blumen N., Munich A., Barkats M., Viollet L.
Ann. Neurol. 71:509-519(2012) [PubMed] [Europe PMC] [Abstract]
Cited for: INVOLVEMENT IN DSMA5.
+Additional computationally mapped references.

Cross-references

Sequence databases

EMBL
GenBank
DDBJ
S37375 mRNA. Translation: AAA09034.1.
S37374 mRNA. Translation: AAA09035.1. Frameshift.
X63368 Genomic DNA. Translation: CAA44968.2.
X63368 Genomic DNA. Translation: CAA44969.2.
BT007088 mRNA. Translation: AAP35751.1.
AK292761 mRNA. Translation: BAF85450.1.
BC011609 mRNA. Translation: AAH11609.1.
BC047056 mRNA. Translation: AAH47056.1.
PIRS23508.
RefSeqNP_001034639.1. NM_001039550.1.
NP_006727.2. NM_006736.5.
UniGeneHs.77768.

3D structure databases

PDBe
RCSB PDB
PDBj
EntryMethodResolution (Å)ChainPositionsPDBsum
2LGWNMR-A1-91[»]
ProteinModelPortalP25686.
SMRP25686. Positions 1-110.
ModBaseSearch...
MobiDBSearch...

Protein-protein interaction databases

BioGrid109533. 15 interactions.
DIPDIP-29051N.
IntActP25686. 5 interactions.
MINTMINT-1192251.
STRING9606.ENSP00000338019.

PTM databases

PhosphoSiteP25686.

Polymorphism databases

DMDM158518384.

Proteomic databases

PaxDbP25686.
PRIDEP25686.

Protocols and materials databases

DNASU3300.
StructuralBiologyKnowledgebaseSearch...

Genome annotation databases

EnsemblENST00000336576; ENSP00000338019; ENSG00000135924. [P25686-3]
ENST00000392086; ENSP00000375936; ENSG00000135924. [P25686-2]
GeneID3300.
KEGGhsa:3300.
UCSCuc002vkw.1. human. [P25686-2]
uc002vkx.1. human. [P25686-3]

Organism-specific databases

CTD3300.
GeneCardsGC02P220143.
HGNCHGNC:5228. DNAJB2.
HPAHPA036268.
MIM604139. gene.
614881. phenotype.
neXtProtNX_P25686.
Orphanet314485. Young adult-onset distal hereditary motor neuropathy.
PharmGKBPA27415.
GenAtlasSearch...

Phylogenomic databases

eggNOGCOG0484.
HOGENOMHOG000111538.
HOVERGENHBG066998.
InParanoidP25686.
KOK09508.
OMARGPRHSG.
OrthoDBEOG7KSX9F.
PhylomeDBP25686.
TreeFamTF105142.

Gene expression databases

ArrayExpressP25686.
BgeeP25686.
CleanExHS_DNAJB2.
GenevestigatorP25686.

Family and domain databases

Gene3D1.10.287.110. 1 hit.
InterProIPR001623. DnaJ_domain.
IPR018253. DnaJ_domain_CS.
IPR003903. Ubiquitin-int_motif.
[Graphical view]
PfamPF00226. DnaJ. 1 hit.
[Graphical view]
PRINTSPR00625. JDOMAIN.
SMARTSM00271. DnaJ. 1 hit.
SM00726. UIM. 2 hits.
[Graphical view]
SUPFAMSSF46565. SSF46565. 1 hit.
PROSITEPS00636. DNAJ_1. 1 hit.
PS50076. DNAJ_2. 1 hit.
PS50330. UIM. 1 hit.
[Graphical view]
ProtoNetSearch...

Other

ChiTaRSDNAJB2. human.
GeneWikiDNAJB2.
GenomeRNAi3300.
NextBio13093.
PROP25686.
SOURCESearch...

Entry information

Entry nameDNJB2_HUMAN
AccessionPrimary (citable) accession number: P25686
Secondary accession number(s): A8K9P6 expand/collapse secondary AC list , Q8IUK1, Q8IUK2, Q96F52
Entry history
Integrated into UniProtKB/Swiss-Prot: May 1, 1992
Last sequence update: September 11, 2007
Last modified: April 16, 2014
This is version 134 of the entry and version 3 of the sequence. [Complete history]
Entry statusReviewed (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.

Relevant documents

SIMILARITY comments

Index of protein domains and families

PDB cross-references

Index of Protein Data Bank (PDB) cross-references

MIM cross-references

Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot

Human polymorphisms and disease mutations

Index of human polymorphisms and disease mutations

Human entries with polymorphisms or disease mutations

List of human entries with polymorphisms or disease mutations

Human chromosome 2

Human chromosome 2: entries, gene names and cross-references to MIM