Hsp40 ImmunoSet
- Known as:
- Hsp40 ImmunoSet
- Catalog number:
- ASA960-073
- Product Quantity:
- 5 x 96 Well
- Category:
- -
- Supplier:
- Other suppliers
- Gene target:
- Hsp40 ImmunoSet
Related genes to: Hsp40 ImmunoSet
- Gene:
- DNAJB1 NIH gene
- Name:
- DnaJ heat shock protein family (Hsp40) member B1
- Previous symbol:
- HSPF1
- Synonyms:
- Hsp40, Sis1, RSPH16B
- Chromosome:
- 19p13.12
- Locus Type:
- gene with protein product
- Date approved:
- 1996-10-02
- Date modifiied:
- 2015-11-19
Related products to: Hsp40 ImmunoSet
ABBP-2,APOBEC1-binding protein 2,DnaJ homolog subfamily B member 11,DnaJ protein homolog 9,DNAJB11,EDJ,ER-associated DNAJ,ER-associated dnaJ protein 3,ER-associated Hsp40 co-chaperone,ERdj3,ERJ3,ERj3pABBP-2,APOBEC1-binding protein 2,DnaJ homolog subfamily B member 11,Dnajb11,ER-associated DNAJ,ER-associated dnaJ protein 3,ER-associated Hsp40 co-chaperone,ERdj3,ERj3p,Mouse,Mus musculusAnti-Hsp40anti-HSP40anti-HSP40anti-HSP40anti-HSP40anti-HSP40Anti-HSp40 (Ydj1)Anti-Hsp40 (Ydj1)anti-HSP40 , Rabbit polyclonal to HSP40 , Isotype IgG, Host RabbitAnti-Hsp40 Antibodyanti-HSP40 type: Primary antibodies host: Mouseanti-HSP40 type: Primary antibodies host: RabbitAnti-HSP40/Hdj1 (DyLight 488 conjugate) Antibody (OAED00142) Related articles to: Hsp40 ImmunoSet
- Fibrolamellar carcinoma (FLC) is a rare liver malignancy affecting adolescents. FLCs harbor a DNAJB1-PRKACA gene fusion that combines heat shock protein DNAJB1 with the catalytic subunit of protein kinase A. Surgery with systemic therapy provides 5-year survivals of 30-50%, but advanced disease remains largely incurable. Three-dimensional explants from 41 FLC patients were interrogated for drug sensitivity, resistance, and synergy against cytotoxics, targeted agents, and signal transduction inhibitors. Sterile specimens from histologically confirmed FLC patients were analyzed by Ex Vivo Analysis of Programmed Cell Death (EVA/PCD™) in a CLIA-licensed laboratory. Following mechanical and enzymatic disaggregation, explants underwent 72 h drug exposure. LC values were derived from five-point dose-response curves and compared with a database of over 10,000 human tumor analyses. Synergy was assessed by combination index. In parallel, targeted metabolomic profiling was performed in five FLC patients using tandem MS/MS. Forty-one samples were analyzed. Of 24 drugs selected, tumor-cell yields were adequate for testing in 18 (75%). Single-agent activity favored vorinostat, followed by phenformin and 6-diazo-5-oxo-L-norleucine. Combinations favored gemcitabine plus oxaliplatin (GEMOX) and 5-FU plus interferon. Metabolomic analysis identified distinct signature consistent with mitochondrial dysfunction and altered polyamine metabolism. The present findings are exploratory, and hypothesis-generating and should not be interpreted as evidence of clinical efficacy. Prospective clinical validation and mechanistic studies will be required to further define the therapeutic relevance of these observations in fibrolamellar carcinoma. - Source: PubMed
Publication date: 2026/05/27
Schneider Sabina AD'Amora PauloEvans Steven SKent PaulStockwell TomBakaya Vikrant SBernard Paula JFrancisco Federico RTorres LuisaHenry JohnSilva Ismael D C GNagourney Robert A - Stress granules are conserved biomolecular condensates that form under stress and rapidly disassemble during recovery. Stress granules have been linked to pathological protein aggregation and their impaired disassembly reduces cell viability, yet the mechanisms governing their clearance and protein aggregation remain unclear. We find that human HSP70 and a subset of J-domain proteins (JDPs) localize to stress granules and that chemical or genetic inhibition of these chaperones markedly slows granule disassembly. Conversely, overexpressing these JDPs, particularly DNAJB1, accelerates disassembly without altering assembly. In vitro, HSP70 and DNAJB1 partition into G3BP1 condensates and reduce their size in an ATP-dependent manner. In cells expressing amyotrophic lateral sclerosis (ALS)-linked mutant FUS, DNAJB1 depletion further impairs stress granule clearance and promotes pre-amyloid accumulation, while depleting a non-stress granule JDP has no effect. Our findings demonstrate that specific JDP chaperones enhance stress granule disassembly and help limit aberrant protein aggregation. - Source: PubMed
Publication date: 2026/06/08
Mastromarco Giovanni JEarnshaw RebeccaMoore GaelenXu X Y SherwinSadek Nour HCui FionaLo NatalieLee Hyun O - Spinocerebellar ataxia type 3 (SCA3), the most common inherited ataxia, lacks effective therapies. Current microRNA (miRNA) approaches directly targeting ataxin-3 (ATXN3) mRNA risk reducing essential wild-type ATXN3, a key regulator of proteostasis and various signaling pathways. Consequently, developing neuron-targeted, noninvasive miRNA delivery strategies that employ alternative mechanisms is critical for SCA3. In this study, we engineered rabies virus glycoprotein (RVG)-modified exosomes to deliver miR-370 inhibitors to the brains of SCA3 mice, thereby upregulating the molecular chaperone DnaJ homolog subfamily B member 1 (DNAJB1) to promote clearance of mutant ATXN3. Systemic administration of RVG-exosomes carrying miR-370 inhibitors led to increased DNAJB1 expression, reduced mutant ATXN3 aggregation, improved neuronal survival, and restored motor coordination in SCA3 mice. Furthermore, combining miR-370 inhibitors with miR-25-a miRNA that directly targets ATXN3 mRNA-synergistically enhanced the reduction of pathogenic ATXN3 and provided greater neuroprotection compared to either therapy alone. These findings support the use of RVG-exosome-mediated miR-370 inhibition as a noninvasive strategy to improve proteostasis in SCA3 and demonstrate its potential in combination with ATXN3 mRNA-targeting miRNAs, offering a promising therapeutic paradigm for SCA3 and similar neurodegenerative disorders. - Source: PubMed
Zhang ZiyiGao ShuguangQin LixiaHe Miao - Intraductal oncocytic papillary neoplasms (IOPNs) are rare tumors that develop from biliary and pancreatic ductal epithelium and progress to lethal cancers. Human IOPNs and IOPN-associated carcinomas are known to harbor the gene fusion, however the role of the oncogenic fusion in carcinogenesis is poorly understood. We developed a Cre-inducible mouse model of human DNAJB1-PRKACA expression and substantiated that the fusion gene is a bona fide driver of IOPN-associated biliary and pancreatic carcinoma. By analyzing the unique histopathologic and transcriptional changes that occur at each stage of tumor development, we found that these murine tumors closely mimic human driven tumors. Furthermore, we identified that many of the salient features of invasive carcinoma are established at the pre-invasive stage, including evidence of tumor cell metabolic dysregulation, immunosuppressive tumor stroma and expression of genes strongly associated with invasive driven cancer in humans. Finally, we found that , a characteristic regulated super-enhancer associated gene, serves as a robust biomarker of malignant transformation from IOPN to invasive carcinoma. - Source: PubMed
Publication date: 2026/05/27
Carney Patrick RNukaya ManabuCarter Jason AVeltri Anthony JMatkowskyj Kristina AStram AustinRubinstein C DustinVeith Alex CPillarisetty Venu GBradfield Christopher ARonnekleiv-Kelly Sean M - Hydrogen-deuterium exchange (HDX) of protein backbone amides provides a powerful probe of conformational dynamics. However, when experiments are performed in HO/DO mixtures, quantitative interpretation is hindered by back exchange and isotope effects not captured by the classical Linderstro̷m-Lang (LL) model. We introduce a generalized Linderstro̷m-Lang (GLL) framework that explicitly accounts for forward and reverse exchange and for changes in protection upon isotopic substitution. Analytical solutions describe equilibrium enrichment (fractionation) and protection factors in mixtures, reducing to the LL model in pure DO. Application to HDX/NMR of the molecular chaperone DNAJB1 in 50% DO demonstrates that the GLL model recovers protection factors at 100% DO. Ignoring back exchange (i.e., using the LL model), protection factors are systematically underestimated. A particularly powerful feature of our approach is that a single HDX experiment in a mixture (e.g., 50% DO) simultaneously provides protection factors that report on conformational dynamics and local stability and fractionation factors that are sensitive to the local hydrogen-bonding environment. - Source: PubMed
Publication date: 2026/03/17
Grimaldi AntonioHobbs BillyStofella MicheleKaramanos Theodoros KPaci Emanuele