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Entry version 199 (10 Feb 2021)
Sequence version 2 (18 Oct 2001)
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Protein

DnaJ homolog subfamily B member 6

Gene

DNAJB6

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

Annotation score:5 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the 'correct annotation' for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
-Experimental evidence at protein leveli <p>This indicates the type of evidence that supports the existence of the protein. Note that the 'protein existence' evidence does not give information on the accuracy or correctness of the sequence(s) displayed.<p><a href='/help/protein_existence' target='_top'>More...</a></p>

<p>This section provides any useful information about the protein, mostly biological knowledge.<p><a href='/help/function_section' target='_top'>More...</a></p>Functioni

Plays an indispensable role in the organization of KRT8/KRT18 filaments. Acts as an endogenous molecular chaperone for neuronal proteins including huntingtin. Suppresses aggregation and toxicity of polyglutamine-containing, aggregation-prone proteins. Isoform B but not isoform A inhibits huntingtin aggregation. Has a stimulatory effect on the ATPase activity of HSP70 in a dose-dependent and time-dependent manner and hence acts as a co-chaperone of HSP70. Also reduces cellular toxicity and caspase-3 activity.5 Publications

<p>The <a href="http://www.geneontology.org/">Gene Ontology (GO)</a> project provides a set of hierarchical controlled vocabulary split into 3 categories:<p><a href='/help/gene_ontology' target='_top'>More...</a></p>GO - Molecular functioni

GO - Biological processi

<p>UniProtKB Keywords constitute a <a href="http://www.uniprot.org/keywords">controlled vocabulary</a> with a hierarchical structure. Keywords summarise the content of a UniProtKB entry and facilitate the search for proteins of interest.<p><a href='/help/keywords' target='_top'>More...</a></p>Keywordsi

Molecular functionChaperone

Enzyme and pathway databases

Pathway Commons web resource for biological pathway data

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PathwayCommonsi
O75190

Reactome - a knowledgebase of biological pathways and processes

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Reactomei
R-HSA-3371453, Regulation of HSF1-mediated heat shock response

<p>This section provides information about the protein and gene name(s) and synonym(s) and about the organism that is the source of the protein sequence.<p><a href='/help/names_and_taxonomy_section' target='_top'>More...</a></p>Names & Taxonomyi

<p>This subsection of the <a href="http://www.uniprot.org/help/names%5Fand%5Ftaxonomy%5Fsection">Names and taxonomy</a> section provides an exhaustive list of all names of the protein, from commonly used to obsolete, to allow unambiguous identification of a protein.<p><a href='/help/protein_names' target='_top'>More...</a></p>Protein namesi
Recommended name:
DnaJ homolog subfamily B member 6
Alternative name(s):
HHDJ1
Heat shock protein J2
Short name:
HSJ-2
MRJ
MSJ-1
<p>This subsection of the <a href="http://www.uniprot.org/help/names%5Fand%5Ftaxonomy%5Fsection">Names and taxonomy</a> section indicates the name(s) of the gene(s) that code for the protein sequence(s) described in the entry. Four distinct tokens exist: 'Name', 'Synonyms', 'Ordered locus names' and 'ORF names'.<p><a href='/help/gene_name' target='_top'>More...</a></p>Gene namesi
Name:DNAJB6
Synonyms:HSJ2, MRJ, MSJ1
<p>This subsection of the <a href="http://www.uniprot.org/help/names%5Fand%5Ftaxonomy%5Fsection">Names and taxonomy</a> section provides information on the name(s) of the organism that is the source of the protein sequence.<p><a href='/help/organism-name' target='_top'>More...</a></p>OrganismiHomo sapiens (Human)
<p>This subsection of the <a href="http://www.uniprot.org/help/names%5Fand%5Ftaxonomy%5Fsection">Names and taxonomy</a> section shows the unique identifier assigned by the NCBI to the source organism of the protein. This is known as the 'taxonomic identifier' or 'taxid'.<p><a href='/help/taxonomic_identifier' target='_top'>More...</a></p>Taxonomic identifieri9606 [NCBI]
<p>This subsection of the <a href="http://www.uniprot.org/help/names%5Fand%5Ftaxonomy%5Fsection">Names and taxonomy</a> section contains the taxonomic hierarchical classification lineage of the source organism. It lists the nodes as they appear top-down in the taxonomic tree, with the more general grouping listed first.<p><a href='/help/taxonomic_lineage' target='_top'>More...</a></p>Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
<p>This subsection of the <a href="http://www.uniprot.org/help/names%5Fand%5Ftaxonomy%5Fsection">Names and taxonomy</a> section is present for entries that are part of a <a href="http://www.uniprot.org/proteomes">proteome</a>, i.e. of a set of proteins thought to be expressed by organisms whose genomes have been completely sequenced.<p><a href='/help/proteomes_manual' target='_top'>More...</a></p>Proteomesi
  • UP000005640 <p>A UniProt <a href="http://www.uniprot.org/manual/proteomes%5Fmanual">proteome</a> can consist of several components.<br></br>The component name refers to the genomic component encoding a set of proteins.<p><a href='/help/proteome_component' target='_top'>More...</a></p> Componenti: Chromosome 7

Organism-specific databases

Human Gene Nomenclature Database

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HGNCi
HGNC:14888, DNAJB6

Online Mendelian Inheritance in Man (OMIM)

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MIMi
611332, gene

neXtProt; the human protein knowledge platform

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neXtProti
NX_O75190

Eukaryotic Pathogen, Vector and Host Database Resources

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VEuPathDBi
HostDB:ENSG00000105993.14

<p>This section provides information on the location and the topology of the mature protein in the cell.<p><a href='/help/subcellular_location_section' target='_top'>More...</a></p>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 & Seán O’Donoghue; Source: COMPARTMENTS

Keywords - Cellular componenti

Cytoplasm, Nucleus

<p>This section provides information on the disease(s) and phenotype(s) associated with a protein.<p><a href='/help/pathology_and_biotech_section' target='_top'>More...</a></p>Pathology & Biotechi

<p>This subsection of the 'Pathology and Biotech' section provides information on the disease(s) associated with genetic variations in a given protein. The information is extracted from the scientific literature and diseases that are also described in the <a href="http://www.ncbi.nlm.nih.gov/sites/entrez?db=omim">OMIM</a> database are represented with a <a href="http://www.uniprot.org/diseases">controlled vocabulary</a> in the following way:<p><a href='/help/involvement_in_disease' target='_top'>More...</a></p>Involvement in diseasei

Muscular dystrophy, limb-girdle, autosomal dominant 1 (LGMDD1)2 Publications
The disease is caused by variants affecting the gene represented in this entry. There is evidence that LGMDD1 is caused by dysfunction of isoform B (PubMed:22366786).1 Publication
Disease descriptionAn autosomal dominant myopathy characterized by adult onset of proximal muscle weakness, beginning in the hip girdle region and later progressing to the shoulder girdle region.
Related information in OMIM
Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the 'Sequence' section describes natural variant(s) of the protein sequence.<p><a href='/help/variant' target='_top'>More...</a></p>Natural variantiVAR_06783389F → I in LGMDD1; the mutation results in inefficient inhibition of protein aggregation by isoform B. 1 PublicationCorresponds to variant dbSNP:rs387907150EnsemblClinVar.1
Natural variantiVAR_06783493F → L in LGMDD1; the mutation results in inefficient inhibition of protein aggregation by isoform B. 2 PublicationsCorresponds to variant dbSNP:rs387907046EnsemblClinVar.1
Natural variantiVAR_06783596P → R in LGMDD1. 1 PublicationCorresponds to variant dbSNP:rs387907047EnsemblClinVar.1

Keywords - Diseasei

Disease variant, Limb-girdle muscular dystrophy

Organism-specific databases

DisGeNET

More...
DisGeNETi
10049

GeneReviews a resource of expert-authored, peer-reviewed disease descriptions.

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GeneReviewsi
DNAJB6

MalaCards human disease database

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MalaCardsi
DNAJB6
MIMi603511, phenotype

Open Targets

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OpenTargetsi
ENSG00000105993

Orphanet; a database dedicated to information on rare diseases and orphan drugs

More...
Orphaneti
34516, DNAJB6-related limb-girdle muscular dystrophy D1

The Pharmacogenetics and Pharmacogenomics Knowledge Base

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PharmGKBi
PA27418

Miscellaneous databases

Pharos NIH Druggable Genome Knowledgebase

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Pharosi
O75190, Tbio

Chemistry databases

ChEMBL database of bioactive drug-like small molecules

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ChEMBLi
CHEMBL4295674

Genetic variation databases

BioMuta curated single-nucleotide variation and disease association database

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BioMutai
DNAJB6

<p>This section describes post-translational modifications (PTMs) and/or processing events.<p><a href='/help/ptm_processing_section' target='_top'>More...</a></p>PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the <a href="http://www.uniprot.org/help/ptm%5Fprocessing%5Fsection">PTM / Processing</a> section indicates that the initiator methionine is cleaved from the mature protein.<p><a href='/help/init_met' target='_top'>More...</a></p>Initiator methionineiRemoved1 Publication
<p>This subsection of the 'PTM / Processing' section describes the extent of a polypeptide chain in the mature protein following processing or proteolytic cleavage.<p><a href='/help/chain' target='_top'>More...</a></p>ChainiPRO_00000710252 – 326DnaJ homolog subfamily B member 6Add BLAST325

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the 'PTM / Processing' section specifies the position and type of each modified residue excluding <a href="http://www.uniprot.org/manual/lipid">lipids</a>, <a href="http://www.uniprot.org/manual/carbohyd">glycans</a> and <a href="http://www.uniprot.org/manual/crosslnk">protein cross-links</a>.<p><a href='/help/mod_res' target='_top'>More...</a></p>Modified residuei135Omega-N-methylarginineBy similarity1
Modified residuei277PhosphoserineCombined sources1

Keywords - PTMi

Methylation, Phosphoprotein

Proteomic databases

Encyclopedia of Proteome Dynamics

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EPDi
O75190

jPOST - Japan Proteome Standard Repository/Database

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jPOSTi
O75190

MassIVE - Mass Spectrometry Interactive Virtual Environment

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MassIVEi
O75190

MaxQB - The MaxQuant DataBase

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MaxQBi
O75190

PaxDb, a database of protein abundance averages across all three domains of life

More...
PaxDbi
O75190

PeptideAtlas

More...
PeptideAtlasi
O75190

PRoteomics IDEntifications database

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PRIDEi
O75190

ProteomicsDB: a multi-organism proteome resource

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ProteomicsDBi
4676
49860 [O75190-1]
49861 [O75190-2]
49862 [O75190-3]

Consortium for Top Down Proteomics

More...
TopDownProteomicsi
O75190-1 [O75190-1]

PTM databases

iPTMnet integrated resource for PTMs in systems biology context

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iPTMneti
O75190

Comprehensive resource for the study of protein post-translational modifications (PTMs) in human, mouse and rat.

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PhosphoSitePlusi
O75190

SwissPalm database of S-palmitoylation events

More...
SwissPalmi
O75190

<p>This section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms.<p><a href='/help/expression_section' target='_top'>More...</a></p>Expressioni

<p>This subsection of the 'Expression' section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms. By default, the information is derived from experiments at the mRNA level, unless specified 'at protein level'.<br></br>Examples: <a href="http://www.uniprot.org/uniprot/P92958#expression">P92958</a>, <a href="http://www.uniprot.org/uniprot/Q8TDN4#expression">Q8TDN4</a>, <a href="http://www.uniprot.org/uniprot/O14734#expression">O14734</a><p><a href='/help/tissue_specificity' target='_top'>More...</a></p>Tissue specificityi

Widely expressed. Highest levels in testis and brain, and lower levels in heart, spleen, intestine, ovary, placenta, lung, kidney, pancreas, thymus, prostate, skeletal muscle, liver and leukocytes. In testis, expressed in germ cells in the earlier stages of differentiation pathway as well as in spermatids. In brain, expressed at a higher level in hippocampus and thalamus and a lower level in amygdala, substantia nigra, corpus callosum and caudate nucleus.4 Publications

Gene expression databases

Bgee dataBase for Gene Expression Evolution

More...
Bgeei
ENSG00000105993, Expressed in cortical plate and 232 other tissues

ExpressionAtlas, Differential and Baseline Expression

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ExpressionAtlasi
O75190, baseline and differential

Genevisible search portal to normalized and curated expression data from Genevestigator

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Genevisiblei
O75190, HS

Organism-specific databases

Human Protein Atlas

More...
HPAi
ENSG00000105993, Low tissue specificity

<p>This section provides information on the quaternary structure of a protein and on interaction(s) with other proteins or protein complexes.<p><a href='/help/interaction_section' target='_top'>More...</a></p>Interactioni

<p>This subsection of the <a href="http://www.uniprot.org/help/interaction%5Fsection">'Interaction'</a> section provides information about the protein quaternary structure and interaction(s) with other proteins or protein complexes (with the exception of physiological receptor-ligand interactions which are annotated in the <a href="http://www.uniprot.org/help/function%5Fsection">'Function'</a> section).<p><a href='/help/subunit_structure' target='_top'>More...</a></p>Subunit structurei

Homooligomer.

Interacts with BAG3, HSPB8 and STUB1.

Interacts with ALKBH1 (By similarity).

Interacts with HSP70, KRT18 and PTTG. Isoform B interacts with histone deacetylases HDAC4, HDAC6, and SIRT2, HDAC activity is required for antiaggregation.

By similarity3 Publications

<p>This subsection of the '<a href="http://www.uniprot.org/help/interaction%5Fsection">Interaction</a>' section provides information about binary protein-protein interactions. The data presented in this section are a quality-filtered subset of binary interactions automatically derived from the <a href="https://www.ebi.ac.uk/intact/">IntAct database</a>. It is updated at every <a href="http://www.uniprot.org/help/synchronization">UniProt release</a>.<p><a href='/help/binary_interactions' target='_top'>More...</a></p>Binary interactionsi

O75190
With#Exp.IntAct
ASCL4 [Q6XD76]3EBI-1053164,EBI-10254793
BRMS1 [Q9HCU9]2EBI-1053164,EBI-714781
CBLB [Q13191]3EBI-1053164,EBI-744027
CFAP100 - isoform 2 [Q494V2-2]3EBI-1053164,EBI-11953200
DYSF [O75923]2EBI-1053164,EBI-2799016
FAM117B [Q6P1L5]3EBI-1053164,EBI-3893327
IK [Q13123]3EBI-1053164,EBI-713456
KRT18 [P05783]6EBI-1053164,EBI-297888
MAGEC3 - isoform 2 [Q8TD91-2]3EBI-1053164,EBI-10694180
MLF1 [P58340]2EBI-1053164,EBI-721328
MLF2 [Q15773]4EBI-1053164,EBI-1051875
RPP25 [Q9BUL9]3EBI-1053164,EBI-366570
SGCG [Q13326]2EBI-1053164,EBI-5357343
SPATA22 [Q8NHS9]3EBI-1053164,EBI-7067260
ZNF829 - isoform 3 [Q3KNS6-3]3EBI-1053164,EBI-18036029
ZSCAN1 - isoform 2 [Q8NBB4-2]3EBI-1053164,EBI-12021938
DNAJB6 - isoform B [O75190-2]
With#Exp.IntAct
AMBN [Q9NP70]3EBI-12593112,EBI-11893530
ANKRD13D - isoform 2 [Q6ZTN6-2]3EBI-12593112,EBI-25840993
ANXA4 [Q6LES2]3EBI-12593112,EBI-10250835
AQP8 [O94778]3EBI-12593112,EBI-19124986
ATP5IF1 [Q9UII2]3EBI-12593112,EBI-718459
ATP6V1B1 [P15313]3EBI-12593112,EBI-2891281
BCAT1 - isoform 4 [P54687-4]3EBI-12593112,EBI-25834445
BCHE [P06276]3EBI-12593112,EBI-7936069
BECN1 [Q14457]3EBI-12593112,EBI-949378
C11orf54 - isoform 3 [Q9H0W9-3]3EBI-12593112,EBI-12108466
CD209 - isoform 10 [Q9NNX6-10]3EBI-12593112,EBI-12300031
CDC23 [Q9UJX2]3EBI-12593112,EBI-396137
CNST - isoform 3 [Q6PJW8-3]3EBI-12593112,EBI-25836090
COX7A1 [P24310]3EBI-12593112,EBI-25876196
CRYBA2 [P53672]3EBI-12593112,EBI-750444
DKK3 [Q9UBP4]3EBI-12593112,EBI-954409
itself2EBI-12593112,EBI-12593112
DOM3Z [A0A024RCP2]3EBI-12593112,EBI-25847826
DPP9 - isoform 2 [Q86TI2-2]3EBI-12593112,EBI-21529239
E4F1 [H3BUJ7]3EBI-12593112,EBI-10178160
FYCO1 - isoform 2 [Q9BQS8-2]3EBI-12593112,EBI-25905795
GINS3 [Q9BRX5]3EBI-12593112,EBI-2857315
H3-5 [Q6NXT2]3EBI-12593112,EBI-2868501
HAUS1 [Q96CS2]3EBI-12593112,EBI-2514791
HAUS7 [Q99871]3EBI-12593112,EBI-395719
HTT [P42858]3EBI-12593112,EBI-466029
IFI35 - isoform 2 [P80217-2]3EBI-12593112,EBI-12823003
IQUB [Q8NA54]3EBI-12593112,EBI-10220600
KIAA0825 [Q8IV33]3EBI-12593112,EBI-17702098
KLC3 [Q6P597]3EBI-12593112,EBI-1643885
KLF5 [Q13887]3EBI-12593112,EBI-2696013
KLHL3 [Q9UH77]3EBI-12593112,EBI-8524663
LOXL4 [Q96JB6]3EBI-12593112,EBI-749562
LURAP1 [Q96LR2]3EBI-12593112,EBI-741355
METTL27 [Q8N6F8]3EBI-12593112,EBI-8487781
MRFAP1 [Q9Y605]3EBI-12593112,EBI-995714
NEFL [I6L9F6]3EBI-12593112,EBI-10178578
OTUB1 [Q96FW1]3EBI-12593112,EBI-1058491
PPP2R1B - isoform 2 [P30154-2]3EBI-12593112,EBI-11058011
RAB3IP - isoform 7 [Q96QF0-7]3EBI-12593112,EBI-11984839
RCN1 [Q15293]3EBI-12593112,EBI-948278
REX1BD [Q96EN9]3EBI-12593112,EBI-745810
SCAPER - isoform 3 [Q9BY12-3]3EBI-12593112,EBI-25837959
SNF8 [Q96H20]3EBI-12593112,EBI-747719
SPATA12 [Q7Z6I5]3EBI-12593112,EBI-10696971
SRGAP3 [Q8IXS7]3EBI-12593112,EBI-18616594
STX11 [O75558]3EBI-12593112,EBI-714135
TCAP [O15273]3EBI-12593112,EBI-954089
THAP3 [Q8WTV1]3EBI-12593112,EBI-17438286
TP53BP1 [Q12888]3EBI-12593112,EBI-396540
TSNAX [Q99598]3EBI-12593112,EBI-742638
TTC33 [Q6PID6]3EBI-12593112,EBI-2555404
VPS11 [Q9H270]3EBI-12593112,EBI-373380
WDR83 [Q9BRX9]3EBI-12593112,EBI-7705033

GO - Molecular functioni

Protein-protein interaction databases

The Biological General Repository for Interaction Datasets (BioGRID)

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BioGRIDi
115360, 101 interactors

Protein interaction database and analysis system

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IntActi
O75190, 144 interactors

Molecular INTeraction database

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MINTi
O75190

STRING: functional protein association networks

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STRINGi
9606.ENSP00000262177

Miscellaneous databases

RNAct, Protein-RNA interaction predictions for model organisms.

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RNActi
O75190, protein

<p>This section provides information on the tertiary and secondary structure of a protein.<p><a href='/help/structure_section' target='_top'>More...</a></p>Structurei

Secondary structure

1326
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details

3D structure databases

SWISS-MODEL Repository - a database of annotated 3D protein structure models

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SMRi
O75190

Database of comparative protein structure models

More...
ModBasei
Search...

Protein Data Bank in Europe - Knowledge Base

More...
PDBe-KBi
Search...

<p>This section provides information on sequence similarities with other proteins and the domain(s) present in a protein.<p><a href='/help/family_and_domains_section' target='_top'>More...</a></p>Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the <a href="http://www.uniprot.org/help/family%5Fand%5Fdomains%5Fsection">Family and Domains</a> section describes the position and type of a domain, which is defined as a specific combination of secondary structures organized into a characteristic three-dimensional structure or fold.<p><a href='/help/domain' target='_top'>More...</a></p>Domaini2 – 69JPROSITE-ProRule annotationAdd BLAST68

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the 'Family and Domains' section describes a region of interest that cannot be described in other subsections.<p><a href='/help/region' target='_top'>More...</a></p>Regioni2 – 146Interaction with HSP701 PublicationAdd BLAST145
Regioni119 – 242Interaction with KRT181 PublicationAdd BLAST124

Compositional bias

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the 'Family and Domains' section describes the position of regions of compositional bias within the protein and the particular amino acids that are over-represented within those regions.<p><a href='/help/compbias' target='_top'>More...</a></p>Compositional biasi83 – 172Gly/Phe-richAdd BLAST90
Compositional biasi155 – 194Ser-richAdd BLAST40

<p>This subsection of the 'Family and domains' section provides general information on the biological role of a domain. The term 'domain' is intended here in its wide acceptation, it may be a structural domain, a transmembrane region or a functional domain. Several domains are described in this subsection.<p><a href='/help/domain_cc' target='_top'>More...</a></p>Domaini

The antiaggregation activity of isoform B resides in the serine-rich region and the C-terminus.

Phylogenomic databases

evolutionary genealogy of genes: Non-supervised Orthologous Groups

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eggNOGi
KOG0714, Eukaryota

Ensembl GeneTree

More...
GeneTreei
ENSGT00940000154205

InParanoid: Eukaryotic Ortholog Groups

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InParanoidi
O75190

Database for complete collections of gene phylogenies

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PhylomeDBi
O75190

TreeFam database of animal gene trees

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TreeFami
TF105142

Family and domain databases

Conserved Domains Database

More...
CDDi
cd06257, DnaJ, 1 hit

Gene3D Structural and Functional Annotation of Protein Families

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Gene3Di
1.10.287.110, 1 hit

Integrated resource of protein families, domains and functional sites

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InterProi
View protein in InterPro
IPR001623, DnaJ_domain
IPR018253, DnaJ_domain_CS
IPR036869, J_dom_sf

Pfam protein domain database

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Pfami
View protein in Pfam
PF00226, DnaJ, 1 hit

Protein Motif fingerprint database; a protein domain database

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PRINTSi
PR00625, JDOMAIN

Simple Modular Architecture Research Tool; a protein domain database

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SMARTi
View protein in SMART
SM00271, DnaJ, 1 hit

Superfamily database of structural and functional annotation

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SUPFAMi
SSF46565, SSF46565, 1 hit

PROSITE; a protein domain and family database

More...
PROSITEi
View protein in PROSITE
PS00636, DNAJ_1, 1 hit
PS50076, DNAJ_2, 1 hit

<p>This section displays by default the canonical protein sequence and upon request all isoforms described in the entry. It also includes information pertinent to the sequence(s), including <a href="http://www.uniprot.org/help/sequence%5Flength">length</a> and <a href="http://www.uniprot.org/help/sequences">molecular weight</a>. The information is filed in different subsections. The current subsections and their content are listed below:<p><a href='/help/sequences_section' target='_top'>More...</a></p>Sequences (4+)i

<p>This subsection of the <a href="http://www.uniprot.org/help/sequences%5Fsection">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical%5Fand%5Fisoforms">canonical sequence</a> displayed by default in the entry is complete or not.<p><a href='/help/sequence_status' target='_top'>More...</a></p>Sequence statusi: Complete.

<p>This subsection of the <a href="http://www.uniprot.org/help/sequences%5Fsection">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical%5Fand%5Fisoforms">canonical sequence</a> displayed by default in the entry is in its mature form or if it represents the precursor.<p><a href='/help/sequence_processing' target='_top'>More...</a></p>Sequence processingi: The displayed sequence is further processed into a mature form.

This entry describes 4 <p>This subsection of the 'Sequence' section lists the alternative protein sequences (isoforms) that can be generated from the same gene by a single or by the combination of up to four biological events (alternative promoter usage, alternative splicing, alternative initiation and ribosomal frameshifting). Additionally, this section gives relevant information on each alternative protein isoform. This section is only present in reviewed entries, i.e. in UniProtKB/Swiss-Prot.<p><a href='/help/alternative_products' target='_top'>More...</a></p> isoformsi produced by alternative splicing. AlignAdd to basket

This entry has 4 described isoforms and 9 potential isoforms that are computationally mapped.Show allAlign All

Isoform A (identifier: O75190-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the <div> <p><b>What is the canonical sequence?</b><p><a href='/help/canonical_and_isoforms' target='_top'>More...</a></p>canonicali 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
MVDYYEVLGV QRHASPEDIK KAYRKLALKW HPDKNPENKE EAERKFKQVA
60 70 80 90 100
EAYEVLSDAK KRDIYDKYGK EGLNGGGGGG SHFDSPFEFG FTFRNPDDVF
110 120 130 140 150
REFFGGRDPF SFDFFEDPFE DFFGNRRGPR GSRSRGTGSF FSAFSGFPSF
160 170 180 190 200
GSGFSSFDTG FTSFGSLGHG GLTSFSSTSF GGSGMGNFKS ISTSTKMVNG
210 220 230 240 250
RKITTKRIVE NGQERVEVEE DGQLKSLTIN GVADDDALAE ERMRRGQNAL
260 270 280 290 300
PAQPAGLRPP KPPRPASLLR HAPHCLSEEE GEQDRPRAPG PWDPLASAAG
310 320
LKEGGKRKKQ KQREESKKKK STKGNH
Length:326
Mass (Da):36,087
Last modified:October 18, 2001 - v2
<p>The checksum is a form of redundancy check that is calculated from the sequence. It is useful for tracking sequence updates.</p> <p>It should be noted that while, in theory, two different sequences could have the same checksum value, the likelihood that this would happen is extremely low.</p> <p>However UniProtKB may contain entries with identical sequences in case of multiple genes (paralogs).</p> <p>The checksum is computed as the sequence 64-bit Cyclic Redundancy Check value (CRC64) using the generator polynomial: x<sup>64</sup> + x<sup>4</sup> + x<sup>3</sup> + x + 1. The algorithm is described in the ISO 3309 standard. </p> <p class="publication">Press W.H., Flannery B.P., Teukolsky S.A. and Vetterling W.T.<br /> <strong>Cyclic redundancy and other checksums</strong><br /> <a href="http://www.nrbook.com/b/bookcpdf.php">Numerical recipes in C 2nd ed., pp896-902, Cambridge University Press (1993)</a>)</p> Checksum:iECF1628BF7A524F3
GO
Isoform B (identifier: O75190-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     232-241: VADDDALAEE → KEQLLRLDNK
     242-326: Missing.

Show »
Length:241
Mass (Da):26,900
Checksum:iCD5A49CAB66C2E16
GO
Isoform C (identifier: O75190-3) [UniParc]FASTAAdd to basket
Also known as: a

The sequence of this isoform differs from the canonical sequence as follows:
     301-326: LKEGGKRKKQKQREESKKKKSTKGNH → VQREAAVEQAQSETSLGARGQRGHK

Show »
Length:325
Mass (Da):35,714
Checksum:i01F47DA9A87EBC74
GO
Isoform D (identifier: O75190-4) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-59: MVDYYEVLGVQRHASPEDIKKAYRKLALKWHPDKNPENKEEAERKFKQVAEAYEVLSDA → MPHPRILKRP

Show »
Length:277
Mass (Da):30,369
Checksum:i97C70A8C1DF2CACC
GO

<p>In eukaryotic reference proteomes, unreviewed entries that are likely to belong to the same gene are computationally mapped, based on gene identifiers from Ensembl, EnsemblGenomes and model organism databases.<p><a href='/help/gene_centric_isoform_mapping' target='_top'>More...</a></p>Computationally mapped potential isoform sequencesi

There are 9 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
A0A0J9YX62A0A0J9YX62_HUMAN
DnaJ homolog subfamily B member 6
DNAJB6
334Annotation score:

Annotation score:2 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the 'correct annotation' for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
E9PH18E9PH18_HUMAN
DnaJ homolog subfamily B member 6
DNAJB6
211Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the 'correct annotation' for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
C9J2C4C9J2C4_HUMAN
DnaJ homolog subfamily B member 6
DNAJB6
228Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the 'correct annotation' for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
C9J2P2C9J2P2_HUMAN
DnaJ homolog subfamily B member 6
DNAJB6
145Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the 'correct annotation' for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
C9JB42C9JB42_HUMAN
DnaJ homolog subfamily B member 6
DNAJB6
66Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the 'correct annotation' for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
C9JDR7C9JDR7_HUMAN
DnaJ homolog subfamily B member 6
DNAJB6
98Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the 'correct annotation' for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
C9JDX6C9JDX6_HUMAN
DnaJ homolog subfamily B member 6
DNAJB6
125Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the 'correct annotation' for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
F8WCZ4F8WCZ4_HUMAN
DnaJ homolog subfamily B member 6
DNAJB6
129Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the 'correct annotation' for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
C9JN01C9JN01_HUMAN
DnaJ homolog subfamily B member 6
DNAJB6
45Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the 'correct annotation' for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>

<p>This subsection of the 'Sequence' section reports difference(s) between the protein sequence shown in the UniProtKB entry and other available protein sequences derived from the same gene.<p><a href='/help/sequence_caution' target='_top'>More...</a></p>Sequence cautioni

The sequence AAD16010 differs from that shown. Reason: Frameshift.Curated
The sequence BAD93096 differs from that shown. Reason: Erroneous initiation.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the 'Sequence' section reports difference(s) between the canonical sequence (displayed by default in the entry) and the different sequence submissions merged in the entry. These various submissions may originate from different sequencing projects, different types of experiments, or different biological samples. Sequence conflicts are usually of unknown origin.<p><a href='/help/conflict' target='_top'>More...</a></p>Sequence conflicti128G → S in BAD97321 (Ref. 9) Curated1
Sequence conflicti303E → G in BAF85714 (PubMed:14702039).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_06783389F → I in LGMDD1; the mutation results in inefficient inhibition of protein aggregation by isoform B. 1 PublicationCorresponds to variant dbSNP:rs387907150EnsemblClinVar.1
Natural variantiVAR_06783493F → L in LGMDD1; the mutation results in inefficient inhibition of protein aggregation by isoform B. 2 PublicationsCorresponds to variant dbSNP:rs387907046EnsemblClinVar.1
Natural variantiVAR_07942896P → L Found in a family with autosomal-dominantly inherited distal-onset myopathy; significant loss of its ability to suppress aggregation of polyglutamine-containing proteins. 1 Publication1
Natural variantiVAR_06783596P → R in LGMDD1. 1 PublicationCorresponds to variant dbSNP:rs387907047EnsemblClinVar.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the 'Sequence' section describes the sequence of naturally occurring alternative protein isoform(s). The changes in the amino acid sequence may be due to alternative splicing, alternative promoter usage, alternative initiation, or ribosomal frameshifting.<p><a href='/help/var_seq' target='_top'>More...</a></p>Alternative sequenceiVSP_0538941 – 59MVDYY…VLSDA → MPHPRILKRP in isoform D. CuratedAdd BLAST59
Alternative sequenceiVSP_001289232 – 241VADDDALAEE → KEQLLRLDNK in isoform B. 10 Publications10
Alternative sequenceiVSP_001290242 – 326Missing in isoform B. 10 PublicationsAdd BLAST85
Alternative sequenceiVSP_026180301 – 326LKEGG…TKGNH → VQREAAVEQAQSETSLGARG QRGHK in isoform C. 4 PublicationsAdd BLAST26

Sequence databases

Select the link destinations:

EMBL nucleotide sequence database

More...
EMBLi

GenBank nucleotide sequence database

More...
GenBanki

DNA Data Bank of Japan; a nucleotide sequence database

More...
DDBJi
Links Updated
AF080569 mRNA Translation: AAD16010.1 Frameshift.
AB015798 mRNA Translation: BAA88769.1
AB015799 mRNA Translation: BAA88770.1
AF060703 mRNA Translation: AAF21257.1
AB014888 mRNA Translation: BAA32209.1
AF075601 mRNA Translation: AAD43194.1
CR533498 mRNA Translation: CAG38529.1
AB209859 mRNA Translation: BAD93096.1 Different initiation.
AK223601 mRNA Translation: BAD97321.1
AK291953 mRNA Translation: BAF84642.1
AK293025 mRNA Translation: BAF85714.1
AK297796 mRNA Translation: BAG60135.1
AL136707 mRNA Translation: CAB66642.1
AC006372 Genomic DNA No translation available.
AC079306 Genomic DNA Translation: AAS07392.1
AC079306 Genomic DNA Translation: AAS07393.1
CH236954 Genomic DNA Translation: EAL23923.1
CH236954 Genomic DNA Translation: EAL23924.1
CH471149 Genomic DNA Translation: EAX04570.1
BC000177 mRNA Translation: AAH00177.1
BC002446 mRNA Translation: AAH02446.1

The Consensus CDS (CCDS) project

More...
CCDSi
CCDS47755.1 [O75190-2]
CCDS5946.1 [O75190-1]

NCBI Reference Sequences

More...
RefSeqi
NP_005485.1, NM_005494.2 [O75190-2]
NP_490647.1, NM_058246.3 [O75190-1]

Genome annotation databases

Ensembl eukaryotic genome annotation project

More...
Ensembli
ENST00000262177; ENSP00000262177; ENSG00000105993 [O75190-1]
ENST00000429029; ENSP00000397556; ENSG00000105993 [O75190-2]

Database of genes from NCBI RefSeq genomes

More...
GeneIDi
10049

KEGG: Kyoto Encyclopedia of Genes and Genomes

More...
KEGGi
hsa:10049

UCSC genome browser

More...
UCSCi
uc003wnj.4, human [O75190-1]

Keywords - Coding sequence diversityi

Alternative splicing

<p>This section provides links to proteins that are similar to the protein sequence(s) described in this entry at different levels of sequence identity thresholds (100%, 90% and 50%) based on their membership in UniProt Reference Clusters (<a href="http://www.uniprot.org/help/uniref">UniRef</a>).<p><a href='/help/similar_proteins_section' target='_top'>More...</a></p>Similar proteinsi

<p>This section is used to point to information related to entries and found in data collections other than UniProtKB.<p><a href='/help/cross_references_section' target='_top'>More...</a></p>Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF080569 mRNA Translation: AAD16010.1 Frameshift.
AB015798 mRNA Translation: BAA88769.1
AB015799 mRNA Translation: BAA88770.1
AF060703 mRNA Translation: AAF21257.1
AB014888 mRNA Translation: BAA32209.1
AF075601 mRNA Translation: AAD43194.1
CR533498 mRNA Translation: CAG38529.1
AB209859 mRNA Translation: BAD93096.1 Different initiation.
AK223601 mRNA Translation: BAD97321.1
AK291953 mRNA Translation: BAF84642.1
AK293025 mRNA Translation: BAF85714.1
AK297796 mRNA Translation: BAG60135.1
AL136707 mRNA Translation: CAB66642.1
AC006372 Genomic DNA No translation available.
AC079306 Genomic DNA Translation: AAS07392.1
AC079306 Genomic DNA Translation: AAS07393.1
CH236954 Genomic DNA Translation: EAL23923.1
CH236954 Genomic DNA Translation: EAL23924.1
CH471149 Genomic DNA Translation: EAX04570.1
BC000177 mRNA Translation: AAH00177.1
BC002446 mRNA Translation: AAH02446.1
CCDSiCCDS47755.1 [O75190-2]
CCDS5946.1 [O75190-1]
RefSeqiNP_005485.1, NM_005494.2 [O75190-2]
NP_490647.1, NM_058246.3 [O75190-1]

3D structure databases

Select the link destinations:

Protein Data Bank Europe

More...
PDBei

Protein Data Bank RCSB

More...
RCSB PDBi

Protein Data Bank Japan

More...
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
6U3RNMR-A1-127[»]
A184-231[»]
6U3SNMR-A1-127[»]
A184-231[»]
SMRiO75190
ModBaseiSearch...
PDBe-KBiSearch...

Protein-protein interaction databases

BioGRIDi115360, 101 interactors
IntActiO75190, 144 interactors
MINTiO75190
STRINGi9606.ENSP00000262177

Chemistry databases

ChEMBLiCHEMBL4295674

PTM databases

iPTMnetiO75190
PhosphoSitePlusiO75190
SwissPalmiO75190

Genetic variation databases

BioMutaiDNAJB6

Proteomic databases

EPDiO75190
jPOSTiO75190
MassIVEiO75190
MaxQBiO75190
PaxDbiO75190
PeptideAtlasiO75190
PRIDEiO75190
ProteomicsDBi4676
49860 [O75190-1]
49861 [O75190-2]
49862 [O75190-3]
TopDownProteomicsiO75190-1 [O75190-1]

Protocols and materials databases

Antibodypedia a portal for validated antibodies

More...
Antibodypediai
3316, 304 antibodies

The DNASU plasmid repository

More...
DNASUi
10049

Genome annotation databases

EnsembliENST00000262177; ENSP00000262177; ENSG00000105993 [O75190-1]
ENST00000429029; ENSP00000397556; ENSG00000105993 [O75190-2]
GeneIDi10049
KEGGihsa:10049
UCSCiuc003wnj.4, human [O75190-1]

Organism-specific databases

Comparative Toxicogenomics Database

More...
CTDi
10049
DisGeNETi10049

GeneCards: human genes, protein and diseases

More...
GeneCardsi
DNAJB6
GeneReviewsiDNAJB6
HGNCiHGNC:14888, DNAJB6
HPAiENSG00000105993, Low tissue specificity
MalaCardsiDNAJB6
MIMi603511, phenotype
611332, gene
neXtProtiNX_O75190
OpenTargetsiENSG00000105993
Orphaneti34516, DNAJB6-related limb-girdle muscular dystrophy D1
PharmGKBiPA27418
VEuPathDBiHostDB:ENSG00000105993.14

GenAtlas: human gene database

More...
GenAtlasi
Search...

Phylogenomic databases

eggNOGiKOG0714, Eukaryota
GeneTreeiENSGT00940000154205
InParanoidiO75190
PhylomeDBiO75190
TreeFamiTF105142

Enzyme and pathway databases

PathwayCommonsiO75190
ReactomeiR-HSA-3371453, Regulation of HSF1-mediated heat shock response

Miscellaneous databases

BioGRID ORCS database of CRISPR phenotype screens

More...
BioGRID-ORCSi
10049, 177 hits in 886 CRISPR screens

ChiTaRS: a database of human, mouse and fruit fly chimeric transcripts and RNA-sequencing data

More...
ChiTaRSi
DNAJB6, human

The Gene Wiki collection of pages on human genes and proteins

More...
GeneWikii
DNAJB6

Database of phenotypes from RNA interference screens in Drosophila and Homo sapiens

More...
GenomeRNAii
10049
PharosiO75190, Tbio

Protein Ontology

More...
PROi
PR:O75190
RNActiO75190, protein

The Stanford Online Universal Resource for Clones and ESTs

More...
SOURCEi
Search...

Gene expression databases

BgeeiENSG00000105993, Expressed in cortical plate and 232 other tissues
ExpressionAtlasiO75190, baseline and differential
GenevisibleiO75190, HS

Family and domain databases

CDDicd06257, DnaJ, 1 hit
Gene3Di1.10.287.110, 1 hit
InterProiView protein in InterPro
IPR001623, DnaJ_domain
IPR018253, DnaJ_domain_CS
IPR036869, J_dom_sf
PfamiView protein in Pfam
PF00226, DnaJ, 1 hit
PRINTSiPR00625, JDOMAIN
SMARTiView protein in SMART
SM00271, DnaJ, 1 hit
SUPFAMiSSF46565, SSF46565, 1 hit
PROSITEiView protein in PROSITE
PS00636, DNAJ_1, 1 hit
PS50076, DNAJ_2, 1 hit

ProtoNet; Automatic hierarchical classification of proteins

More...
ProtoNeti
Search...

MobiDB: a database of protein disorder and mobility annotations

More...
MobiDBi
Search...

<p>This section provides general information on the entry.<p><a href='/help/entry_information_section' target='_top'>More...</a></p>Entry informationi

<p>This subsection of the 'Entry information' section provides a mnemonic identifier for a UniProtKB entry, but it is not a stable identifier. Each reviewed entry is assigned a unique entry name upon integration into UniProtKB/Swiss-Prot.<p><a href='/help/entry_name' target='_top'>More...</a></p>Entry nameiDNJB6_HUMAN
<p>This subsection of the 'Entry information' section provides one or more accession number(s). These are stable identifiers and should be used to cite UniProtKB entries. Upon integration into UniProtKB, each entry is assigned a unique accession number, which is called 'Primary (citable) accession number'.<p><a href='/help/accession_numbers' target='_top'>More...</a></p>AccessioniPrimary (citable) accession number: O75190
Secondary accession number(s): A4D232
, A8K7D8, A8KAG0, B4DN73, E9PCZ2, O95806, Q53EN8, Q59EF2, Q6FIC8, Q75MA2, Q9UIK6
<p>This subsection of the 'Entry information' section shows the date of integration of the entry into UniProtKB, the date of the last sequence update and the date of the last annotation modification ('Last modified'). The version number for both the entry and the <a href="http://www.uniprot.org/help/canonical%5Fand%5Fisoforms">canonical sequence</a> are also displayed.<p><a href='/help/entry_history' target='_top'>More...</a></p>Entry historyiIntegrated into UniProtKB/Swiss-Prot: May 30, 2000
Last sequence update: October 18, 2001
Last modified: February 10, 2021
This is version 199 of the entry and version 2 of the sequence. See complete history.
<p>This subsection of the 'Entry information' section indicates whether the entry has been manually annotated and reviewed by UniProtKB curators or not, in other words, if the entry belongs to the Swiss-Prot section of UniProtKB (<strong>reviewed</strong>) or to the computer-annotated TrEMBL section (<strong>unreviewed</strong>).<p><a href='/help/entry_status' target='_top'>More...</a></p>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.

<p>This section contains any relevant information that doesn't fit in any other defined sections<p><a href='/help/miscellaneous_section' target='_top'>More...</a></p>Miscellaneousi

Keywords - Technical termi

3D-structure, Direct protein sequencing, Reference proteome

Documents

  1. Human variants curated from literature reports
    Index of human variants curated from literature reports
  2. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  3. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  4. Human chromosome 7
    Human chromosome 7: entries, gene names and cross-references to MIM
  5. Human entries with genetic variants
    List of human entries with genetic variants
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