Affinage

DNAJB6

DnaJ homolog subfamily B member 6 · UniProt O75190

Length
326 aa
Mass
36.1 kDa
Annotated
2026-06-09
93 papers in source corpus 48 papers cited in narrative 49 extracted findings
Cross-family judge vs UniProt: Affinage preferred faithfulness: 6/6 claims corpus-supported (100%)

Mechanistic narrative

Synthesis pass · prose summary of the discoveries below

DNAJB6 is an oligomeric HSP40/J-domain co-chaperone that functions as a generalist suppressor of pathological protein aggregation and phase transitions across development, muscle, and neurons (PMID:11896048, PMID:23904097, PMID:36302971). It directly binds aggregation-prone substrates at substoichiometric ratios — polyglutamine peptides, amyloid-β42, and α-synuclein — through conserved S/T residues in a C-terminal peptide-binding cleft formed at its oligomeric dimer interface, blocking primary and secondary nucleation of amyloid fibrils (PMID:23904097, PMID:25217638, PMID:30024736, PMID:29581438, PMID:32350108). Anti-aggregation activity for some clients (e.g. α-synuclein) requires the J domain and Hsp70 partnership and routes misfolded species toward proteasomal degradation, whereas direct fibrillation suppression of polyQ and Aβ proceeds independently of ATP and Hsp70 (PMID:23904097, PMID:28831037, PMID:31514384). The protein exists as cytoplasmic (DNAJB6b) and nuclear (DNAJB6a) isoforms with distinct roles: the cytoplasmic isoform surveils FG-nucleoporin phase transitions to support interphase nuclear pore complex biogenesis and locks FUS condensates into a non-fibrillar gel-like state (PMID:36302971, PMID:41271702), while the nuclear isoform restrains tumor progression by assembling Hsp70/PP2A complexes that activate GSK3β and by suppressing Wnt/β-catenin and AKT signaling (PMID:22266849, PMID:22455953, PMID:26302489). In development, DNAJB6 prevents cytotoxic keratin-18 aggregation required for chorioallantoic fusion and supports neural stem cell self-renewal (PMID:17409114, PMID:19777589). Dominant missense mutations in the G/F and J domains cause limb-girdle muscular dystrophy (LGMDD1); these mutations disrupt a G/F-domain helical element that normally regulates Hsp70 binding, producing unregulated Hsp70 hyperactivation and depletion and impairing CASA/BAG3-dependent sarcomeric protein quality control, rather than a simple loss of anti-aggregation activity (PMID:22366786, PMID:26847086, PMID:37923706).

Mechanistic history

Synthesis pass · year-by-year structured walk · 16 steps
  1. 1999 High

    Established DNAJB6 as an essential developmental gene before any molecular activity was known, anchoring its in vivo importance.

    Evidence Gene-trap null allele in mouse with embryological analysis of placental development

    PMID:10021343

    Open questions at the time
    • Did not define the molecular activity underlying the chorioallantoic fusion defect
    • Did not distinguish isoform-specific contributions
  2. 2000 High

    Defined DNAJB6 as a substrate-specific co-chaperone, linking its J domain to Hsp70 and its C-terminus to a client (keratin 18) for cytoskeletal organization.

    Evidence Yeast two-hybrid, reciprocal Co-IP, and anti-Mrj antibody microinjection in HeLa cells

    PMID:10954706

    Open questions at the time
    • Did not resolve whether keratin binding reflects folding or degradation triage
    • No structural basis for C-terminal client binding
  3. 2007 High

    Resolved the developmental mechanism: DNAJB6 prevents cytotoxic keratin aggregation rather than maintaining normal keratin, established by genetic epistasis.

    Evidence Mrj−/− × K18-null double-mutant rescue and proteasome inhibition in mouse placenta

    PMID:17409114

    Open questions at the time
    • Did not show direct biochemical disaggregation of keratin in vitro
    • Aggregate-clearance route only inferred from proteasome dependence
  4. 2006 Medium

    Extended anti-aggregation function to neurodegeneration-relevant polyQ proteins and showed it acts by raising soluble monomeric client, distinct from aggregation-promoting J proteins.

    Evidence Cell-based HD aggregation assay and Drosophila polyQ neurodegeneration model with colocalization

    PMID:11896048 PMID:16934481

    Open questions at the time
    • Mechanism of solubilization not biochemically defined at this stage
    • Overexpression-based; endogenous requirement not tested
  5. 2008 High

    Revealed DNAJB6 as a regulator of client nuclear import and stability, and uncovered an isoform/localization dimension with opposing cancer outcomes.

    Evidence Co-IP, knockdown/transgenic T-cell models for Slfn1 import; ectopic large-isoform expression in breast cancer with secretome MS

    PMID:18328103 PMID:18373498

    Open questions at the time
    • Did not unify how chaperone activity drives transcriptional/secretome changes
    • Isoform-specific structural determinants of localization unresolved
  6. 2012 High

    Identified DNAJB6 G/F-domain mutations as the cause of LGMD1D and tied disease specifically to the cytoplasmic isoform with reduced anti-aggregation activity and CASA/BAG3 association.

    Evidence Patient exome sequencing, Drosophila and cell functional tests, in vitro aggregation assay, Co-IP with CASA members; replicated with TDP-43 muscle pathology

    PMID:22334415 PMID:22366786

    Open questions at the time
    • Initial framing as loss of anti-aggregation activity (later revised)
    • Half-life extension mechanism not structurally explained at this point
  7. 2012 High

    Defined a nuclear/cancer signaling axis in which DNAJB6 assembles an Hsp70-PP2A complex to activate GSK3β and drives a β-catenin/MSX1/DKK1 feedback loop to suppress EMT and metastasis.

    Evidence Co-IP of multiprotein complex, J-domain deletion, GSK3β phospho readouts, ChIP/reporter assays, tumor assays

    PMID:22266849 PMID:22455953

    Open questions at the time
    • Whether co-chaperone activity per se versus scaffolding drives signaling not separated
    • Connection between aggregation surveillance and transcriptional control unclear
  8. 2013 High

    Demonstrated that DNAJB6 is an oligomer that directly and substoichiometrically suppresses amyloid fibrillation independently of ATP and Hsp70, defining an intrinsic anti-aggregation mechanism.

    Evidence Purified-protein thioflavin T fibrillation assays with polyQ45, ATP/HSPA1 independence tests, comparison to dimeric DNAJB1; parallel cellular polyQ assays

    PMID:23612975 PMID:23904097

    Open questions at the time
    • Did not localize the substrate-binding surface on the protein
    • Oligomer architecture not yet resolved
  9. 2014 High

    Established that DNAJB6 targets aggregated/oligomeric Aβ species and inhibits both primary and secondary nucleation, becoming incorporated into fibrils.

    Evidence Thioflavin T kinetics, CD, quantitative kinetic analysis, immunochemistry with purified Aβ42; triple complex Co-IP with Hsp70/uPAR in cancer cells

    PMID:25175595 PMID:25217638

    Open questions at the time
    • Residues mediating Aβ binding not identified
    • Stoichiometry of fibril incorporation not quantified
  10. 2018 High

    Mapped the molecular basis of anti-aggregation to conserved S/T residues lining a peptide-binding cleft at the oligomer dimer interface.

    Evidence Systematic S/T-to-A mutagenesis with SPR, microscale thermophoresis, NMR; crosslinking MS, SAXS, and negative-stain EM of oligomers

    PMID:29581438 PMID:30024736

    Open questions at the time
    • High-resolution structure of the full oligomer not obtained
    • How dynamic subunit exchange couples to client capture unresolved
  11. 2017 High

    Showed J-domain/Hsp70-dependent handling of α-synuclein and that endogenous DNAJB6 is required to prevent aggregation, defining a degradation-coupled route distinct from ATP-independent fibrillation blockade.

    Evidence CRISPR KO with rescue, catalytically dead H31Q J-domain mutant, and MG132 proteasome inhibition in α-syn cell models

    PMID:28831037 PMID:31514384

    Open questions at the time
    • Why some clients require Hsp70 while others do not is not mechanistically unified
    • In vivo relevance shown only later
  12. 2019 High

    Connected DNAJB6 to muscle and cytoskeletal physiology through GSK3β-dependent sarcomeric proteostasis, mitotic spindle function, and a J-domain disease class with reduced disaggregation activity.

    Evidence CRISPR KO myoblasts with quantitative proteomics and LGMD1D mouse lithium rescue; RanGTP-dependent p150Glued Co-IP and spindle assays; J-domain mutation filter-trap/TDP-43 disaggregation assays

    PMID:31064815 PMID:31123706 PMID:31955980

    Open questions at the time
    • Direct substrate at the sarcomere not biochemically identified
    • Mechanistic link between chaperone activity and GSK3β regulation in muscle incomplete
  13. 2020 High

    Identified oligomeric Aβ as the captured species by native MS and explained neuronal vulnerability via developmental loss of DNAJB6 expression.

    Evidence Native mass spectrometry with WT vs S/T mutant DNAJB6; iPSC-derived neuron differentiation with knockdown/overexpression rescue and parallel CRISPR comparison to DNAJA1/DNAJB1

    PMID:32268123 PMID:32350108 PMID:32424160

    Open questions at the time
    • Why DNAJA1 and DNAJB6 act oppositely on polyQ not fully explained
    • Transcriptional control of neuronal DNAJB6 downregulation unresolved
  14. 2022 High

    Defined a cell-biological surveillance role at nuclear pore complexes and refined the disease mechanism toward dominant-negative substrate/Hsp70-cycle perturbation.

    Evidence Immunoelectron tomography, in vitro FG-Nup aggregation assays and Co-IP for NPC biogenesis; yeast Sis1 prion model with ATPase-cycle and dimerization assays for LGMDD1 mutants

    PMID:35931773 PMID:36302971

    Open questions at the time
    • How FG-Nup surveillance is spatially restricted to NPC intermediates not fully defined
    • Reconciliation of substrate-specific versus Hsp70-cycle disease effects pending
  15. 2023 High

    Resolved the LGMDD1 mechanism as a toxic gain of function: mutations disrupt a G/F-domain helix regulating Hsp70 binding, causing unregulated Hsp70 hyperactivation and depletion that is reversible by interfering with the DNAJB6–Hsp70 interaction.

    Evidence Solution NMR structural characterization, in vitro aggregation and Hsp70 interaction assays, myocyte Hsp70 measurement, disease phenotype reversal; FBXL21 ubiquitination of DNAJB6/Desmin (preprint)

    PMID:37797698 PMID:37923706 PMID:42239455

    Open questions at the time
    • FBXL21 regulation reported only in preprint without independent confirmation
    • Therapeutic window for Hsp70-interaction disruption in vivo not established
  16. 2025 High

    Generalized DNAJB6 function to control of biomolecular condensates, showing it co-phase-separates with FUS and FG-Nups to maintain non-fibrillar gel-like states via its intrinsically disordered region.

    Evidence Biophysical co-phase-separation and deep mutational scanning with FUS-ALS mouse model; FG-Nup phase transition assays with IDR mutants (preprint) and NMR of GF-linker autoinhibition (preprint)

    PMID:41271702

    Open questions at the time
    • IDR phase-control mechanism and GF-linker autoinhibition reported partly in preprints awaiting peer review
    • How condensate control integrates with classical Hsp70 co-chaperone cycling unresolved

Open questions

Synthesis pass · forward-looking unresolved questions
  • It remains unresolved how DNAJB6's intrinsic, Hsp70-independent anti-aggregation activity is mechanistically coordinated with its Hsp70-dependent, degradation-coupled and signaling roles, and how isoform localization and oligomer dynamics partition these functions across tissues.
  • No high-resolution structure of a client-bound oligomer
  • No unified model linking condensate control, Hsp70 cycling, and transcriptional/signaling outputs
  • Isoform-specific structural switch governing nuclear vs cytoplasmic function undefined

Mechanism profile

Synthesis pass · controlled-vocabulary classification · explore literature graph →
Molecular activity
GO:0044183 protein folding chaperone 5 GO:0098772 molecular function regulator activity 4 GO:0140313 molecular sequestering activity 4 GO:0140096 catalytic activity, acting on a protein 3
Localization
GO:0005634 nucleus 4 GO:0005829 cytosol 3 GO:0005635 nuclear envelope 1 GO:0005815 microtubule organizing center 1
Pathway
R-HSA-162582 Signal Transduction 4 R-HSA-8953897 Cellular responses to stimuli 4 R-HSA-1266738 Developmental Biology 3 R-HSA-392499 Metabolism of proteins 3 R-HSA-1852241 Organelle biogenesis and maintenance 1
Partners
AKT1BAG3DCTN1FBXL21HSPA8KRT18NFATC3PLAUR
Complex memberships
CASA/BAG3 complexDNAJB6–HSP70–uPAR complexDNAJB6–HSPA8–PP2A complex

Evidence

Reading pass · 49 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
1999 Mrj (DNAJB6) is essential for chorioallantoic fusion in mouse placental development; null homozygous mutants die at mid-gestation due to failure of this process, with reduced expression of trophoblast-specific transcription factors Err2 and Gcm1 in the chorion. Gene-trap null allele in mouse; embryological analysis of Mrj−/− conceptuses Development High 10021343
2000 DNAJB6 (Mrj) directly binds keratin 18 (K18) through its C-terminus and interacts with Hsp/c70 via its J domain; microinjection of anti-Mrj antibody disorganizes K8/18 intermediate filaments without affecting actin or microtubules, indicating Mrj is required for K8/18 filament organization as a K18-specific co-chaperone. Yeast two-hybrid, co-immunoprecipitation, immunostaining, anti-Mrj antibody microinjection in HeLa cells The Journal of Biological Chemistry High 10954706
2002 DNAJB6 (MRJ) suppresses polyglutamine-dependent protein aggregation, caspase activation, and cellular toxicity in a cell model of Huntington's disease; MRJ is highly enriched in the CNS. Cell-based aggregation assay (in vitro HD model), overexpression of MRJ in cells, caspase activity assay The Journal of Biological Chemistry Medium 11896048
2005 DNAJB6 (Mrj) directly associates with NFATc3 and recruits class II histone deacetylases (HDACs) to repress NFAT transcriptional activity in the nucleus; this requires heat shock stimulation, reduces NFATc3 occupancy at the TNF-α promoter, and blocks calcineurin-induced cardiomyocyte hypertrophic growth. siRNA-mediated knockdown of Mrj augments NFAT activity and induces cardiomyocyte hypertrophy. Yeast two-hybrid screen, Co-IP, in vitro binding assay, siRNA knockdown, chromatin immunoprecipitation (ChIP), reporter assays Molecular and Cellular Biology High 16260608
2006 Drosophila MRJ (dmrj) ortholog of human DNAJB6 suppresses polyglutamine toxicity in neurons and co-localizes with polyQ inclusions; it increases the level of detergent-soluble monomeric polyQ-expanded proteins, a different mechanism from dHDJ1 which promotes cytoplasmic aggregation. Drosophila transgenic overexpression, eye pigmentation/structural integrity assay, quantitative PCR, colocalization imaging Neurobiology of Disease Medium 16934481
2007 In Mrj (DNAJB6)-null mouse placenta, absence of Mrj prevents proteasome-dependent degradation of keratin 18 (K18), causing keratin inclusion body formation in chorionic trophoblasts that disrupts chorioallantoic attachment. Genetic reduction of keratin expression in Mrj−/− conceptuses rescues chorioallantoic attachment, demonstrating keratin aggregate cytotoxicity—not loss of normal keratin—is the pathological mechanism. Genetic rescue experiments (Mrj−/− crossed to K18-null), proteasome inhibitor treatment, mouse knockout analysis Development High 17409114
2008 DNAJB6 enhances nuclear import of Schlafen1 (Slfn1) and stabilizes it in complex with Hsp70; in DnaJB6 knockdown cells, Slfn1 is sequestered in the cytoplasm and no cell-cycle arrest occurs. Transgenic DnaJB6 expression in T-lineage cells promotes Slfn1 nuclear import, inhibits Slfn1 degradation, downregulates cyclin D1, and suppresses T-cell proliferation. Co-IP, knockdown/overexpression in T-cell lines, subcellular fractionation, FACS cell cycle analysis, DnaJB6 transgenic mice The Biochemical Journal High 18373498
2008 DNAJB6 large isoform (MRJ-L) contains a functional nuclear localization sequence and when expressed in breast cancer cells reduces migration, invasion, and orthotopic tumor growth; the secreted proteome of MRJ(L)-expressing cells shows reduced osteopontin (SPP1), SPARC, and NPM1 and increased KiSS1. Ectopic expression in breast cancer cell lines, in vivo nude mouse orthotopic tumor model, mass spectrometry of secreted proteome, NLS identification Breast Cancer Research Medium 18328103
2009 DNAJB6 is present in the core of Lewy bodies in Parkinson's disease substantia nigra and cortex, suggesting early involvement of this chaperone in the neuronal disease process; DNAJB6 is strongly upregulated in parkinsonian astrocytes. Immunohistochemistry of post-mortem PD brain tissue with confocal imaging; in silico transcriptome analysis Journal of Neuroscience Research Medium 18711724
2009 Mrj is required for neural stem cell self-renewal; Mrj−/− neurospheres are significantly smaller and form fewer secondary neurospheres. Loss of Mrj causes neural tube defects and reduces proliferating neuroepithelial cells and expression of progenitor markers (Pax6, Olig2, Hes5) independently of the placental phenotype. Conditional rescue experiments separating neural from placental phenotype, neurosphere assay, BrdU proliferation analysis, molecular marker expression Developmental Dynamics Medium 19777589
2010 DNAJB6 large isoform (MRJ-L) induces upregulation of DKK1 (a Wnt/β-catenin signaling inhibitor), causing degradation of β-catenin and downregulation of mesenchymal markers (vimentin, N-cadherin, Twist, Slug) with upregulation of keratin 18, leading to partial reversal of mesenchymal phenotype in cancer cells. Ectopic expression of MRJ(L) in cancer cell lines, Western blot, immunostaining, morphological analysis The Journal of Biological Chemistry Medium 20522561
2010 DNAJB6 (MRJ) interacts with uPAR (urokinase receptor) via its C-terminal region; this complex enhances uPAR-mediated cell adhesion to vitronectin. Full-length MRJ is required for interaction and adhesion enhancement; truncated N- or C-terminal constructs alone are insufficient. Yeast two-hybrid screen, GST pulldown, co-immunoprecipitation, cell adhesion assay, deletion mapping International Journal of Oncology Medium 20372789
2011 Mrj-null trophoblast cells accumulate keratin aggregates, causing collapse of actin cytoskeleton, E-cadherin and β-catenin misexpression, ECM disorganization, and failure to adhere and differentiate into syncytiotrophoblast. Plating Mrj-deficient cells on exogenous laminin-511 normalizes their behavior. Mrj−/− mouse trophoblast cell analysis, immunofluorescence, in vitro differentiation assay, laminin rescue experiment Developmental Dynamics Medium 21972064
2012 Dominant missense mutations in the G/F domain of DNAJB6 (Phe89Ile, Phe93Leu, Phe93Ile) cause LGMD1D. Functional testing in vivo showed mutations have a dominant toxic effect mediated specifically by the cytoplasmic isoform (DNAJB6b). In vitro studies showed mutations increase DNAJB6 half-life, extend this effect to the wild-type protein, and reduce its anti-aggregation activity. DNAJB6 interacts with members of the CASA complex including BAG3. Patient exome sequencing, in vivo Drosophila and cell functional tests, in vitro aggregation assay, co-immunoprecipitation with CASA complex members Nature Genetics High 22366786
2012 DNAJB6 mutations in the G/F domain (Phe93Leu, Pro96Arg) cause dominant myopathy with abnormal aggregation of TDP-43 and DNAJB6 itself in affected muscle, consistent with impaired anti-aggregation chaperone function. Exome sequencing, linkage analysis, muscle histochemistry and immunohistochemistry for TDP-43 and DNAJB6 Annals of Neurology Medium 22334415
2012 DNAJB6 binds HSPA8 (HSC70) via its J domain and recruits protein phosphatase PP2A to dephosphorylate GSK3β at Ser9, thereby activating GSK3β, promoting β-catenin degradation, reducing TCF/LEF activity, and suppressing osteopontin (OPN) expression. Deletion of the J domain abolishes assembly of this multiprotein complex. Co-immunoprecipitation of DNAJB6–HSPA8–PP2A complex, Western blot for GSK3β phosphorylation, deletion mutagenesis of J domain, in vitro and in vivo tumor assays Oncogene High 22266849
2012 DNAJB6 suppresses tumor metastasis and EMT by upregulating DKK1 transcription. Mechanistically, β-catenin stabilization (from DNAJB6 silencing) drives MSX1 transcription, which then suppresses DKK1 at its promoter. This defines a β-catenin/MSX1/DKK1 negative feedback loop regulated by DNAJB6. siRNA knockdown, ChIP assay on MSX1 and DKK1 promoters, reporter assays, overexpression studies The Biochemical Journal Medium 22455953
2012 miR-632 targets the coding region of DNAJB6, downregulates DNAJB6 protein levels, and increases invasive ability of breast cancer cells. Silencing endogenous miR-632 abrogates invasive ability and promotes epithelial characteristics. miRNA target prediction, luciferase reporter assay, miRNA overexpression and silencing, invasion assay Laboratory Investigation Medium 22710984
2012 MRJ(S) (cytoplasmic DNAJB6b) translocates to the nucleus in response to heat shock and hypoxia via a NLS-independent mechanism mediated by a 20-amino-acid C-terminal stress-sensing region; constitutive nuclear localization of MRJ(S) promotes proliferation and invasiveness. Deletion analysis, stress induction experiments, subcellular fractionation and imaging Experimental Cell Research Medium 22504047
2013 Purified DNAJB6 forms large heterogeneous oligomers (unlike dimeric DNAJB1) and, at substoichiometric molar ratios, directly suppresses fibrillation of polyQ45 peptides in vitro independently of HSPA1 and ATP. The suppression is a direct protein-protein interaction between DNAJB6 and polyQ peptides. Protein purification, thioflavin T fibrillation assay in vitro, comparison with DNAJB1, ATP/HSPA1 independence tests Cell Stress & Chaperones High 23904097
2013 DNAJB6 and DNAJB8, but not HSPA/Hsp70 or DNAJB1, prevent intracellular aggregation of polyglutamine peptides expressed in cells; DNAJB6 and DNAJB8 affect soluble polyQ peptide levels, indicating direct inhibition of polyQ peptide aggregation (not via indirect cellular effects). Cellular polyQ peptide aggregation assay, fluorescence microscopy, filter trap assay The Journal of Biological Chemistry High 23612975
2014 DNAJB6 inhibits amyloid-β42 (Aβ42) fibrillation at highly sub-stoichiometric molar ratios by interacting with aggregated (not monomeric) forms of Aβ42, preventing growth of aggregated species and inhibiting both primary and secondary nucleation. DNAJB6 is gradually incorporated into growing fibrils and depleted from solution. Thioflavin T fluorescence, far-UV CD spectroscopy, quantitative kinetic analysis, immunochemistry The Journal of Biological Chemistry High 25217638
2014 DNAJB6 (MRJ) forms a triple complex with HSP70 and uPAR; MRJ overexpression enhances HSP70–uPAR interaction while MRJ knockdown reduces soluble uPAR and triple complex formation. Knockdown of HSP70 and/or MRJ inhibits uPAR-mediated cell adhesion, invasion, migration, and MMP2/MMP9 expression via the MAPK/ERK and FAK pathways. Co-immunoprecipitation, overexpression, siRNA knockdown, transwell invasion assay, wound-healing assay, Western blot BMC Cancer Medium 25175595
2015 The J domain HPD motif of DNAJB6a is required for its tumor-suppressive effects; DNAJB6a forms a complex with AKT1 in living cells (detected by bimolecular fluorescence complementation) and reduces AKT signaling. Loss of DNAJB6a results in upregulation of AKT signaling; nuclear DNAJB6 localization is required for its anti-proliferative effects in cancer cells. Bimolecular fluorescence complementation, xenograft tumor assay, immunoblot of AKT signaling, shRNA knockdown, NLS mutant overexpression Gastroenterology High 26302489
2016 DNAJB6b (long isoform, dnajb6b(L)) acts as a cardioprotective gene in zebrafish and mouse cardiomyopathy models; loss-of-function exacerbates cardiomyopathy, and the deleterious effects are ameliorated by inhibition of ER stress. Overexpression of dnajb6(L) exerts cardioprotective effects. Zebrafish insertional mutagenesis screen, doxorubicin stress assay, ER stress inhibitor treatment, mouse cardiomyopathy model JCI Insight Medium 27642634
2016 Overexpression of DNAJB6 in cytoplasmic inclusions of LGMD1D muscle is associated with markers of defective chaperone-assisted selective autophagy (CASA) including BAG3, ubiquitin, TDP-43, p62, and SMI-31, indicating DNAJB6 mutations impair CASA-dependent protein quality control at the sarcomere. Immunohistochemistry, electron microscopy, extensive protein marker panel on LGMD1D patient muscle biopsies Acta Neuropathologica Communications Medium 26847086
2017 DNAJB6 suppression of α-synuclein aggregation requires its J domain (H31Q catalytically inactive mutant cannot suppress aggregation) and is dependent on Hsp70 (the co-chaperone partnership). CRISPR/Cas9 knockout of DNAJB6 in HEK293T-α-syn cells causes massive α-syn aggregation, reversed by DNAJB6 re-introduction. CRISPR/Cas9 KO, re-introduction with J-domain mutant (H31Q), fluorescence microscopy for aggregation Scientific Reports High 28831037
2018 Conserved S/T residues in the unique S/T-rich region of DNAJB6 are required for efficient inhibition of Aβ42 amyloid fibril formation; progressive S/T-to-A substitutions progressively reduce suppression, particularly of primary nucleation kinetics. S/T residues mediate binding to Aβ42 (measured by SPR and microscale thermophoresis), and DNAJB6 keeps monomeric Aβ42 soluble over extended time. Thioflavin T fibrillation assay, surface plasmon resonance, microscale thermophoresis, NMR spectroscopy, systematic S/T substitution mutagenesis Biochemistry High 30024736
2018 The DNAJB6 oligomeric structure includes a peptide-binding cleft lined with conserved S/T residues at the dimer interface; oligomers are dynamic and exchange subunits. Elongated particles (160×120 Å) were detected by EM, and crosslinking MS provided distance constraints supporting a dimer model. Lysine-specific crosslinking MS, homology modeling, docking, mixed isotope crosslinking, SAXS, negative-stain EM with single particle reconstruction Scientific Reports High 29581438
2018 Morpholino-mediated isoform switching of DNAJB6/MRJ (reducing MRJ-L relative to MRJ-S) suppresses HIV-1 and RSV replication; CstF64 reduction correlates with increase of MRJ-L in macrophages. MRJ-L depletion reduced viral mRNA/genome production. Morpholino oligonucleotide treatment, viral replication assay (RT-qPCR for viral genome), CstF64 manipulation Molecular Therapy. Nucleic Acids Medium 30641477
2019 DNAJB6 mutations in the J domain (p.A50V, p.E54A) cause dominant distal/proximo-distal myopathy with histology similar to G/F domain mutations; both J-domain mutations show reduced anti-aggregation capacity by filter trap assay and TDP-43 disaggregation assays. Structural modeling shows mutated J-domain residues are in close proximity to G/F domain residues. Targeted gene sequencing, filter trap assay, TDP-43 disaggregation assay, structural protein modeling Neuromuscular Disorders Medium 31955980
2019 DNAJB6 CRISPR knockout in HEK293T-α-syn cells increases seeded α-syn aggregation induced by recombinant preformed fibrils (PFFs); this increase is strongly reduced by proteasomal inhibitor MG132, suggesting DNAJB6 targets aggregated/misfolded α-syn for proteasomal degradation. DNAJB6b but not DNAJB1 suppresses seeded α-syn aggregation. CRISPR/Cas9 KO, α-syn PFF seeding assay, fluorescence microscopy and FRET analysis, proteasome inhibitor (MG132) treatment International Journal of Molecular Sciences Medium 31514384
2019 Hsp40 DNAJB6 interacts with JEV NS3 protein (identified by yeast two-hybrid and confirmed by co-localization and Co-IP); loss of DNAJB6 function increases JEV replication without affecting viral binding or internalization, indicating DNAJB6 negatively regulates post-entry viral replication. Yeast two-hybrid screen, co-immunoprecipitation, co-localization imaging, DNAJB6 knockout/knockdown with viral replication assay International Journal of Molecular Sciences Medium 31739611
2019 DNAJB6 knockout in myoblasts leads to accumulation of sarcomeric proteins and hypertrophic myotubes with enhanced fusion index, correlating with diminished GSK3β activity. In contrast, LGMD1D DNAJB6 mutations (e.g., F93L) enhance GSK3β activation and suppress β-catenin and NFAT3c signaling. GSK3β inhibition with lithium chloride improves muscle size and strength in a DNAJB6b-F93L LGMD1D mouse model. CRISPR/Cas9 KO myoblasts, stable isotope labeling/quantitative mass spectrometry, histochemistry, immunohistochemistry, grip strength and inverted wire hang tests in LGMD1D mouse model, lithium chloride treatment Neurology. Genetics High 31123706
2019 DnaJB6 is a RanGTP-regulated protein that interacts with dynactin subunit p150Glued (DCTN1) in a RanGTP-dependent manner specifically during M-phase; it promotes spindle pole focusing and dynein force generation during mitosis. Co-immunoprecipitation (M-phase specific), RanGTP dependency assay, spindle pole focusing analysis, dynein force generation assay Journal of Cell Science Medium 31064815
2020 Loss of DNAJB6 expression during neuronal differentiation (confirmed in iPSC-derived neurons and in vivo) explains neuronal hypersensitivity to polyQ aggregation in SCA3 and HD. DNAJB6 upregulation in neurons antagonizes glutamate-induced polyQ aggregation; DNAJB6 knockdown in neural progenitors causes spontaneous polyQ aggregation. iPSC generation and neuronal differentiation, glutamate treatment aggregation assay, DNAJB6 overexpression and siRNA knockdown in neurons/progenitors, in vivo expression confirmation Molecular Cell High 32268123
2020 DNAJB6 directly captures oligomeric forms of Aβ (Aβ1-40) via its S/T residues, as detected by native mass spectrometry; WT DNAJB6 reduces signals from Aβ oligomers and appears to form dimers/trimers bound to Aβ, while S/T-to-A mutant does not. This confirms oligomeric Aβ (not monomers) as the primary DNAJB6 substrate for inhibiting primary nucleation. Native mass spectrometry-based detection of Aβ oligomers with WT vs S/T mutant DNAJB6 The Journal of Biological Chemistry High 32350108
2020 DNAJB6 knockout in HEK293 cells causes a 5-fold increase in polyQ74-huntingtin aggregation and increased cell death; DNAJA1 KO causes a 4-fold decrease in aggregation, demonstrating that DNAJA1 and DNAJB6 modulate polyQ aggregation in opposite directions. DNAJB1 KO had no effect. CRISPR/Cas9 KO of DNAJB6, DNAJA1, DNAJB1, fluorescence microscopy, filter trap assay, trypan blue and PI cell death assays Scientific Reports High 32424160
2021 Overexpression of DNAJB6b in rat substantia nigra via AAV suppresses α-syn aggregation induced by α-syn overexpression, strongly reduces dopaminergic cell death, and rescues motor behavior deficits (stepping test) in an in vivo Parkinson's disease model. AAV6-mediated overexpression in rat SNpc, α-syn PFF seeding cell assay, immunohistochemistry for dopaminergic neurons, behavioral assessment Neurobiology of Disease High 34390836
2022 DNAJB6 forms foci near nuclear pore complexes (NPCs) and localizes inside herniations at NPC biogenesis intermediates (confirmed by immunoelectron tomography). DNAJB6 binds FG-Nups and prevents aggregation of FG regions in cells and in vitro. Loss of DNAJB6 causes accumulation of cytosolic annulate lamellae, demonstrating a role in interphase NPC biogenesis quality control. Immunoelectron tomography, live imaging, in vitro FG-Nup aggregation assay, DNAJB6 knockout with NPC biogenesis readout, Co-IP of FG-Nups Nature Cell Biology High 36302971
2022 Disease-associated LGMDD1 mutations in DNAJB6 G/F domain alter function in a substrate/conformer-specific manner: they change the structure of client aggregates, interfere with the Hsp70 ATPase cycle, reduce dimerization, and impair substrate processing in a dominant-negative manner (poisoning WT Sis1 function in yeast prion model). Yeast prion model (Sis1 as functional homolog), in vitro chaperone activity assays, Hsp70 ATPase cycle assay, dimerization analysis Nature Communications High 35931773
2022 The T193A mutation in the C-terminal domain (CTD) of DNAJB6 reduces self-oligomerization and anti-aggregation activity. NMR shows the mutation has minimal effects on β-stranded CTD structure but increases population and rate of a partially folded state via β-strand peptide plane flips at ≈100 μs timescale, altering ability to oligomerize. NMR spectroscopy including relaxation-based methods, oligomerization assays Angewandte Chemie High 35247211 38505697
2022 Dnajb6 is required for cardiac pacemaker function; GBT411 zebrafish insertional mutant (trapping Dnajb6) shows sick sinus syndrome (SSS)-like cardiac arrhythmia. Dnajb6 is expressed in a subpopulation of sinus node pacemaker cells overlapping with HCN4 expression. Zebrafish insertional mutagenesis (gene-break transposon), electrocardiographic measurement, in situ expression analysis, mouse model validation eLife Medium 36255053
2023 LGMDD1 disease-mutant DNAJB6 proteins show no reduction in aggregation-prevention activity in vitro; instead, solution NMR reveals structural changes in the G/F domain that disrupt regulation of Hsp70 binding. While WT DNAJB6 contains a helical element that regulates its ability to activate Hsp70, LGMDD1 mutants lack this regulation and hyperactivate Hsp70 in an unregulated manner, depleting Hsp70 in myocytes. Interfering with DNAJB6–Hsp70 binding reverses the disease phenotype. Solution NMR (structural characterization), in vitro aggregation assay, biochemical Hsp70 interaction assays, myocyte Hsp70 level measurement, disease phenotype reversal experiment Nature Communications High 37923706
2023 The C-terminal domain (CTD) of DNAJB6 (specifically the first two β-strands) is sufficient to inhibit secondary nucleation of Aβ42 fibril formation by binding Aβ42 fibrils, but inhibition of primary nucleation requires the full-length protein including regions outside the CTD. The S/T residues in the CTD are required for CTD dimerization/stability but not for secondary nucleation inhibition per se. CTD constructs grafted onto scaffold protein, thioflavin T kinetics, native MS, chemical crosslinking, surface plasmon resonance The Journal of Biological Chemistry High 37797698
2023 FBXL21 ubiquitinates DNAJB6 and its client protein Desmin for proteasomal degradation; LGMDD1 DNAJB6 mutations render resistance to FBXL21-directed degradation. Fbxl21 KO causes aberrant Desmin accumulation and aggravated cytoplasmic TDP-43 accumulation during heat shock. Ubiquitination assay, proteasomal degradation assay, Fbxl21 KO cells, LGMDD1 mutation functional test, TDP-43 localization under stress bioRxivpreprint Medium 42239455
2025 DNAJB6 co-phase separates with FUS-containing condensates and locks them into a loose gel-like state that prevents fibrilization, thereby suppressing FUS-ALS toxicity. Domain mapping and deep mutational scan identify key residues required for this activity. DNAJB6 overexpression prevents motor neuron loss and microglial activation in a mouse model of FUS-ALS. Yeast multiplex genetic screen, biophysical examination of co-phase separation, domain mapping, deep mutational scan, mouse FUS-ALS model with motor neuron counting Nature Communications High 41271702
2025 The conserved intrinsically disordered region (IDR) of DNAJB6 promotes stable gel-like assemblies that prevent aberrant phase transitions of FG-Nups; DNAJB6, DNAJB2, and DNAJB8 all prevent FG-Nup aberrant phase transitions. Mutant analysis shows the sequence space for the IDR is narrow and optimized to avoid self-aggregation while providing anti-amyloidogenic capacity. Phase separation assay for FG-Nups, DNAJB6 IDR mutant analysis, gel-like assembly characterization bioRxivpreprint Medium
2025 The GF-linker of DNAJB6 (as with DNAJB1) creates a hydrophobic partially collapsed cluster with the J-domain that provides autoinhibition of Hsp70 binding; disruption of this cluster destabilizes autoinhibition. The GF-linker is also recognized by the substrate-binding domain of Hsc70 and dictates the lifetime of the JDP–Hsc70 complex; both functions are DNAJB-class member-specific. NMR spectroscopy of autoinhibited DNAJB6 constructs, allosteric communication analysis, Hsc70 binding assays bioRxivpreprint Medium

Source papers

Stage 0 corpus · 93 papers · ranked by NIH iCite citations
Year Title Journal Citations PMID
2012 Mutations affecting the cytoplasmic functions of the co-chaperone DNAJB6 cause limb-girdle muscular dystrophy. Nature genetics 215 22366786
2014 Interaction of the molecular chaperone DNAJB6 with growing amyloid-beta 42 (Aβ42) aggregates leads to sub-stoichiometric inhibition of amyloid formation. The Journal of biological chemistry 151 25217638
2012 Exome sequencing reveals DNAJB6 mutations in dominantly-inherited myopathy. Annals of neurology 139 22334415
2019 CircPLEKHM3 acts as a tumor suppressor through regulation of the miR-9/BRCA1/DNAJB6/KLF4/AKT1 axis in ovarian cancer. Molecular cancer 123 31623606
2013 The DNAJB6 and DNAJB8 protein chaperones prevent intracellular aggregation of polyglutamine peptides. The Journal of biological chemistry 121 23612975
2013 DNAJB6 is a peptide-binding chaperone which can suppress amyloid fibrillation of polyglutamine peptides at substoichiometric molar ratios. Cell stress & chaperones 99 23904097
1999 Mrj encodes a DnaJ-related co-chaperone that is essential for murine placental development. Development (Cambridge, England) 97 10021343
2002 Characterization of a brain-enriched chaperone, MRJ, that inhibits Huntingtin aggregation and toxicity independently. The Journal of biological chemistry 96 11896048
2005 The DnaJ-related factor Mrj interacts with nuclear factor of activated T cells c3 and mediates transcriptional repression through class II histone deacetylase recruitment. Molecular and cellular biology 86 16260608
2017 The molecular chaperones DNAJB6 and Hsp70 cooperate to suppress α-synuclein aggregation. Scientific reports 82 28831037
2008 Large isoform of MRJ (DNAJB6) reduces malignant activity of breast cancer. Breast cancer research : BCR 81 18328103
2000 Identification of Mrj, a DnaJ/Hsp40 family protein, as a keratin 8/18 filament regulatory protein. The Journal of biological chemistry 80 10954706
2020 DNAJB6, a Key Factor in Neuronal Sensitivity to Amyloidogenesis. Molecular cell 66 32268123
2009 DnaJB6 is present in the core of Lewy bodies and is highly up-regulated in parkinsonian astrocytes. Journal of neuroscience research 59 18711724
2015 Nuclear Localization of DNAJB6 Is Associated With Survival of Patients With Esophageal Cancer and Reduces AKT Signaling and Proliferation of Cancer Cells. Gastroenterology 54 26302489
2018 Conserved S/T Residues of the Human Chaperone DNAJB6 Are Required for Effective Inhibition of Aβ42 Amyloid Fibril Formation. Biochemistry 53 30024736
2012 DNAJB6 chaperones PP2A mediated dephosphorylation of GSK3β to downregulate β-catenin transcription target, osteopontin. Oncogene 53 22266849
2010 DNAJB6 induces degradation of beta-catenin and causes partial reversal of mesenchymal phenotype. The Journal of biological chemistry 51 20522561
2007 The Mrj co-chaperone mediates keratin turnover and prevents the formation of toxic inclusion bodies in trophoblast cells of the placenta. Development (Cambridge, England) 50 17409114
2019 DNAJB6 Promotes Ferroptosis in Esophageal Squamous Cell Carcinoma. Digestive diseases and sciences 49 31701262
2013 DNAJB6 myopathy in an Asian cohort and cytoplasmic/nuclear inclusions. Neuromuscular disorders : NMD 47 23394708
2008 The Hsp40 family chaperone protein DnaJB6 enhances Schlafen1 nuclear localization which is critical for promotion of cell-cycle arrest in T-cells. The Biochemical journal 47 18373498
2020 Amyloid-β oligomers are captured by the DNAJB6 chaperone: Direct detection of interactions that can prevent primary nucleation. The Journal of biological chemistry 44 32350108
2020 Co-chaperones DNAJA1 and DNAJB6 are critical for regulation of polyglutamine aggregation. Scientific reports 44 32424160
2018 Structural modelling of the DNAJB6 oligomeric chaperone shows a peptide-binding cleft lined with conserved S/T-residues at the dimer interface. Scientific reports 44 29581438
2015 Complete loss of the DNAJB6 G/F domain and novel missense mutations cause distal-onset DNAJB6 myopathy. Acta neuropathologica communications 44 26205529
2022 The chaperone DNAJB6 surveils FG-nucleoporins and is required for interphase nuclear pore complex biogenesis. Nature cell biology 43 36302971
2006 A Drosophila ortholog of the human MRJ modulates polyglutamine toxicity and aggregation. Neurobiology of disease 40 16934481
2012 DNAJB6 governs a novel regulatory loop determining Wnt/β-catenin signalling activity. The Biochemical journal 39 22455953
2019 The Molecular Chaperone DNAJB6, but Not DNAJB1, Suppresses the Seeded Aggregation of Alpha-Synuclein in Cells. International journal of molecular sciences 38 31514384
2016 A modifier screen identifies DNAJB6 as a cardiomyopathy susceptibility gene. JCI insight 38 27642634
2016 Diagnostically important muscle pathology in DNAJB6 mutated LGMD1D. Acta neuropathologica communications 35 26847086
2014 Exome sequencing identifies a DNAJB6 mutation in a family with dominantly-inherited limb-girdle muscular dystrophy. Neuromuscular disorders : NMD 33 24594375
2015 Novel mutations in DNAJB6 gene cause a very severe early-onset limb-girdle muscular dystrophy 1D disease. Neuromuscular disorders : NMD 32 26338452
2023 DNAJB6 mutants display toxic gain of function through unregulated interaction with Hsp70 chaperones. Nature communications 31 37923706
2021 DNAJB6 suppresses alpha-synuclein induced pathology in an animal model of Parkinson's disease. Neurobiology of disease 31 34390836
2016 Emerging roles and underlying molecular mechanisms of DNAJB6 in cancer. Oncotarget 31 27276715
2016 DNAJB6 Myopathies: Focused Review on an Emerging and Expanding Group of Myopathies. Frontiers in molecular biosciences 31 27747217
2012 Micro-RNA-632 downregulates DNAJB6 in breast cancer. Laboratory investigation; a journal of technical methods and pathology 31 22710984
2014 Overexpression of DNAJB6 promotes colorectal cancer cell invasion through an IQGAP1/ERK-dependent signaling pathway. Molecular carcinogenesis 30 25044025
2013 'Pathognomonic' muscle imaging findings in DNAJB6 mutated LGMD1D. European journal of neurology 29 23865856
2016 Genetic interaction of hnRNPA2B1 and DNAJB6 in a Drosophila model of multisystem proteinopathy. Human molecular genetics 26 26744327
2019 Mutations in the J domain of DNAJB6 cause dominant distal myopathy. Neuromuscular disorders : NMD 25 31955980
2014 Heat shock proteins HSP70 and MRJ cooperatively regulate cell adhesion and migration through urokinase receptor. BMC cancer 25 25175595
2009 Neural stem cell self-renewal requires the Mrj co-chaperone. Developmental dynamics : an official publication of the American Association of Anatomists 25 19777589
1999 Cloning, tissue expression, and chromosomal assignment of human MRJ gene for a member of the DNAJ protein family. Journal of human genetics 25 10319584
2018 Astrocytic expression of the chaperone DNAJB6 results in non-cell autonomous protection in Huntington's disease. Neurobiology of disease 24 30408590
2015 A novel mutation in DNAJB6, p.(Phe91Leu), in childhood-onset LGMD1D with a severe phenotype. Neuromuscular disorders : NMD 24 26371419
2011 Cell-cell adhesion defects in Mrj mutant trophoblast cells are associated with failure to pattern the chorion during early placental development. Developmental dynamics : an official publication of the American Association of Anatomists 23 21972064
2019 Hsp40 Protein DNAJB6 Interacts with Viral NS3 and Inhibits the Replication of the Japanese Encephalitis Virus. International journal of molecular sciences 22 31739611
2018 Novel mutations in DNAJB6 cause LGMD1D and distal myopathy in French families. European journal of neurology 22 29437287
2019 The Host Heat Shock Protein MRJ/DNAJB6 Modulates Virus Infection. Frontiers in microbiology 20 31921062
2015 Evaluation of the amyloid beta-GFP fusion protein as a model of amyloid beta peptides-mediated aggregation: a study of DNAJB6 chaperone. Frontiers in molecular neuroscience 19 26283911
2014 DNAJB6 myopathy: a vacuolar myopathy with childhood onset. Muscle & nerve 19 24170373
2010 Interaction between urokinase receptor and heat shock protein MRJ enhances cell adhesion. International journal of oncology 18 20372789
2023 The C-terminal domain of the antiamyloid chaperone DNAJB6 binds to amyloid-β peptide fibrils and inhibits secondary nucleation. The Journal of biological chemistry 17 37797698
2022 Microsecond Backbone Motions Modulate the Oligomerization of the DNAJB6 Chaperone. Angewandte Chemie (International ed. in English) 17 35247211
2017 A novel DNAJB6 mutation causes dominantly inherited distal-onset myopathy and compromises DNAJB6 function. Clinical genetics 17 28233300
2011 Four new Finnish families with LGMD1D; refinement of the clinical phenotype and the linked 7q36 locus. Neuromuscular disorders : NMD 17 21376592
2019 Lithium chloride corrects weakness and myopathology in a preclinical model of LGMD1D. Neurology. Genetics 15 31123706
2018 Interference of DNAJB6/MRJ Isoform Switch by Morpholino Inhibits Replication of HIV-1 and RSV. Molecular therapy. Nucleic acids 15 30641477
2008 Cell cycle specific expression and nucleolar localization of human J-domain containing co-chaperone Mrj. Molecular and cellular biochemistry 13 19002655
2022 A phenotype-based forward genetic screen identifies Dnajb6 as a sick sinus syndrome gene. eLife 12 36255053
2018 LGMD1D myopathy with cytoplasmic and nuclear inclusions in a Saudi family due to DNAJB6 mutation. Acta myologica : myopathies and cardiomyopathies : official journal of the Mediterranean Society of Myology 12 30838352
2023 miR-632 Induces DNAJB6 Inhibition Stimulating Endothelial-to-Mesenchymal Transition and Fibrosis in Marfan Syndrome Aortopathy. International journal of molecular sciences 11 37894814
2020 Porcine DNAJB6 promotes PCV2 replication via enhancing the formation of autophagy in host cells. Veterinary research 11 32381067
2019 Intrafamilial variability of limb-girdle muscular dystrophy, LGMD1D type. European journal of medical genetics 10 31034989
2018 Human DnaJB6 Antiamyloid Chaperone Protects Yeast from Polyglutamine Toxicity Separately from Spatial Segregation of Aggregates. Molecular and cellular biology 10 30224519
2015 Cell and Context-Dependent Effects of the Heat Shock Protein DNAJB6 on Neuronal Survival. Molecular neurobiology 9 26476842
2023 DNAJB6 isoform specific knockdown: Therapeutic potential for limb girdle muscular dystrophy D1. Molecular therapy. Nucleic acids 8 37346979
2019 DnaJB6 is a RanGTP-regulated protein required for microtubule organization during mitosis. Journal of cell science 8 31064815
2018 Two Korean Families with Limb-Girdle Muscular Dystrophy Type 1D Associated with DNAJB6 Mutations. Yonsei medical journal 8 29869469
2012 Cellular stress stimulates nuclear localization signal (NLS) independent nuclear transport of MRJ. Experimental cell research 8 22504047
2022 Disease-associated mutations within the yeast DNAJB6 homolog Sis1 slow conformer-specific substrate processing and can be corrected by the modulation of nucleotide exchange factors. Nature communications 7 35931773
2020 Long non-coding RNA Hsp4 alleviates lipopolysaccharide-induced apoptosis of lung epithelial cells via miRNA-466m-3p/DNAjb6 axis. Experimental and molecular pathology 7 32976821
2024 Human milk exosome-derived circDNAJB6 improves bronchopulmonary dysplasia model by promoting DNAJB6 gene transcription. Journal of bioenergetics and biomembranes 5 38244155
2024 Mrj is a chaperone of the Hsp40 family that regulates Orb2 oligomerization and long-term memory in Drosophila. PLoS biology 5 38648719
2024 DNAJB6 is expressed in neurons and oligodendrocytes of the human brain. Glia 5 39228066
2022 DNAJB6-Containing Extracellular Vesicles as Chaperone Delivery Systems: A Proteomic Analysis. Pharmaceutics 5 36432676
2017 The Short Isoform of DNAJB6 Protects against 1-Methyl-4-phenylpridinium Ion-Induced Apoptosis in LN18 Cells via Inhibiting Both ROS Formation and Mitochondrial Membrane Potential Loss. Oxidative medicine and cellular longevity 5 28280525
2017 Characteristic Posterior-dominant Lower Limb Muscle Involvement in Limb-girdle Muscular Dystrophy due to a DNAJB6 Phe93Leu Mutation. Internal medicine (Tokyo, Japan) 4 28794355
2023 MUC1 promotes lymph node metastasis in esophageal squamous cell carcinoma by downregulating DNAJB6 expression. Environmental toxicology 3 37584547
2025 DNAJB6: A guardian against neurodegeneration. Neural regeneration research 2 40536953
2024 Abnormally High Expression of DNAJB6 Accelerates Malignant Progression of Lung Adenocarcinoma. Biomedicines 2 39335495
2022 Case Report: A Novel Splice-Site Mutation in DNAJB6 Associated With Juvenile-Onset Proximal-Distal Myopathy in a Chinese Patient. Frontiers in genetics 2 35812750
2025 Dual targeting of the Wnt and DNAJB6/MRJ regulatory loop as an anti-RSV strategy. Virology 1 40737980
2025 Multiplex neurodegeneration proteotoxicity platform reveals DNAJB6 promotes non-toxic FUS condensate gelation and inhibits neurotoxicity. Nature communications 1 41271702
2022 A female carrier of spinal and bulbar muscular atrophy diagnosed with DNAJB6-related distal myopathy. Journal of human genetics 1 35165376
2022 Human J-Domain Protein DnaJB6 Protects Yeast from [PSI+] Prion Toxicity. Biology 1 36552355
2022 Microsecond Backbone Motions Modulate the Oligomerization of the DNAJB6 Chaperone. Angewandte Chemie (Weinheim an der Bergstrasse, Germany) 1 38505697
2016 Heat shock protein HSP40 family chaperone DNAJB6/MRJ expression analysis in blood cells obtained from patients with atopic dermatitis in different phases. Patologicheskaia fiziologiia i eksperimental'naia terapiia 1 29244458
2026 Novel cell-based assay enables FRET-based measurements of the dimerization activity of the chaperone DNAJB6. Biology methods & protocols 0 41717343
2026 FBXL21 regulates diurnal proteostasis and stress response by targeting DNAJB6 and client proteins. bioRxiv : the preprint server for biology 0 42239455

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