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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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DNAJB9
DnaJ heat shock protein family (Hsp40) member B9
Chromosome 7 · 7q31.1|14q24.2-q24.3
NCBI Gene: 4189Ensembl: ENSG00000128590.6HGNC: HGNC:6968UniProt: Q6FIF1
64PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
ERAD pathwayprotein bindingendoplasmic reticulummisfolded protein bindinginsomniapolycythemiatrauma complicationpulmonary vascular congestion
✦AI Summary

DNAJB9 is an endoplasmic reticulum (ER)-luminal co-chaperone of heat shock protein 70 (HSPA5/BiP) that functions as a key regulator of cellular protein quality control and stress responses. Mechanistically, DNAJB9 represses the IRE1-mediated unfolded protein response (UPR) by recruiting BiP to prevent dimerization and activation of the ER stress sensor ERN1/IRE1 1. Additionally, DNAJB9 participates in endoplasmic reticulum-associated degradation (ERAD), facilitating clearance of misfolded proteins including the cystic fibrosis transmembrane conductance regulator (CFTR); DNAJB9 knockdown increases surface expression of both wild-type and ΔF508-CFTR 2. Beyond ER homeostasis, DNAJB9 inhibits p53-dependent oncogene-induced senescence, promoting neoplastic transformation through direct p53 interaction 3. Clinically, DNAJB9 has emerged as a highly specific and sensitive biomarker (98% sensitivity, 99% specificity) for diagnosing fibrillary glomerulonephritis, a rare renal disease characterized by 12-24 nm fibrils in glomeruli 45. DNAJB9 expression is upregulated in response to various cellular stressors, including cadmium exposure and single-walled carbon nanotubes 67. Furthermore, elevated DNAJB9 levels associate with accelerated aging phenotypes and increased risk of age-related diseases in socially disadvantaged populations 8.

Sources cited
1
DNAJB9 involvement in UPR and ERAD crosstalk through IRE1α regulation
PMID: 30056836
2
DNAJB9 is an ER luminal co-chaperone that interacts with CFTR and promotes its ERAD; knockdown increases surface expression of WT and ΔF508-CFTR
PMID: 31285458
3
DNAJB9 inhibits p53-dependent oncogene-induced senescence and induces cell transformation through direct p53 interaction
PMID: 32264658
4
DNAJB9 is a specific and sensitive biomarker (98% sensitivity, 99% specificity) for fibrillary glomerulonephritis diagnosis; role in pathogenesis remains unclear
PMID: 35369552
5
Anti-DNAJB9 immunohistochemistry has revolutionized FGN diagnosis with excellent sensitivity and specificity, superseding electron microscopy
PMID: 38755072
6
DNAJB9 is upregulated in hepatocellular carcinoma cells following chronic cadmium exposure and is classified as an anti-apoptotic J protein
PMID: 26314618
7
DNAJB9 expression is upregulated by single-walled carbon nanotubes in normal human astrocytes in dose-dependent manner, associated with ER stress
PMID: 37561833
8
DNAJB9 is one of 14 age-related proteins elevated in socially disadvantaged individuals, mediating up to 39% of associations with 66 age-related diseases
PMID: 40087516
Disease Associationsⓘ20
insomniaOpen Targets
0.14Weak
polycythemiaOpen Targets
0.14Weak
trauma complicationOpen Targets
0.13Weak
crush injuryOpen Targets
0.12Weak
ovarian dysfunctionOpen Targets
0.12Weak
pulmonary vascular congestionOpen Targets
0.12Weak
myopiaOpen Targets
0.11Weak
exostosisOpen Targets
0.09Suggestive
type 1 diabetes mellitusOpen Targets
0.08Suggestive
breast cancerOpen Targets
0.08Suggestive
triple-negative breast cancerOpen Targets
0.06Suggestive
musculoskeletal system diseaseOpen Targets
0.04Suggestive
hyperinsulinism due to HNF1A deficiencyOpen Targets
0.04Suggestive
cystic fibrosisOpen Targets
0.04Suggestive
hyperinsulinemic hypoglycemia, familial, 2Open Targets
0.03Suggestive
exercise-induced hyperinsulinismOpen Targets
0.03Suggestive
hypoinsulinemic hypoglycemia and body hemihypertrophyOpen Targets
0.03Suggestive
autosomal dominant hyperinsulinism due to Kir6.2 deficiencyOpen Targets
0.03Suggestive
autosomal dominant hyperinsulinism due to SUR1 deficiencyOpen Targets
0.03Suggestive
coffee consumptionOpen Targets
0.03Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
ATF6Protein interaction100%EDEM1Protein interaction98%ERN1Protein interaction95%XBP1Protein interaction95%SIL1Protein interaction95%HYOU1Protein interaction94%
Tissue Expression6 tissues
Liver
100%
Heart
70%
Bone Marrow
55%
Brain
39%
Lung
30%
Ovary
25%
Gene Interaction Network
Click a node to explore
DNAJB9ATF6EDEM1ERN1XBP1SIL1HYOU1
PROTEIN STRUCTURE
Preparing viewer…
PDB2CTR · NMR
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.96LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.59 [0.38–0.96]
RankingsWhere DNAJB9 stands among ~20K protein-coding genes
  • #7,259of 20,598
    Most Researched64
  • #9,114of 17,882
    Most Constrained (LOEUF)0.96
Genes detectedDNAJB9
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Social disadvantage accelerates aging.
PMID: 40087516
Nat Med · 2025
1.00
2
Quality Control in the Endoplasmic Reticulum: Crosstalk between ERAD and UPR pathways.
PMID: 30056836
Trends Biochem Sci · 2018
0.90
3
[Fibrillary glomerulonephritis].
PMID: 38755072
Rev Med Interne · 2024
0.80
4
Expression of DNAJB9 and some other genes is more sensitive to SWCNTs in normal human astrocytes than glioblastoma cells.
PMID: 37561833
Endocr Regul · 2023
0.70
5
Fibrillary Glomerulonephritis and DnaJ Homolog Subfamily B Member 9 (DNAJB9).
PMID: 35369552
Kidney360 · 2020
0.60