The ALS‐associated E425K mutation uncouples DNAJC7 from the Hsp70 chaperone cycle
DNAJC7 is a J‐domain protein that plays a key role in protein quality control by regulating Hsp70 activity and preventing protein aggregation. We find that the ALS‐associated E425K mutation in DNAJC7 disrupts productive interaction and activation of Hsp70, thereby blocking the transfer and refolding of client proteins such as TDP‐43.
Bar Elmaleh +2 more
wiley +1 more source
An Hsp70 Chaperone Is Involved in Meiotic Silencing by Unpaired DNA. [PDF]
Sy VT +3 more
europepmc +1 more source
Here, we report the role of extracellular vesicles (EVs) released from melanoma cells constitutively expressing MHC class II in the regulation of immune cell functions and melanoma metastasis. In particular, we observed an increased localization of HLA‐DRα, CAM receptors, PD‐L1, and STAT3 signaling proteins in the EVs.
Francesca Costantini +4 more
wiley +1 more source
Divergent roles of Hsp70 chaperones in orthoflavivirus protein secretion and virion formation. [PDF]
Blank L, Lorenz C, Steffen I.
europepmc +1 more source
Proteostasis ensures proper protein folding, modification, and degradation, while its impairment triggers ER stress. Chronic ER stress and maladaptive UPR via the CHOP–ERO1 axis remodel ERMCs, altering calcium signaling and mitochondrial metabolism.
Giorgia Maria Renna +5 more
wiley +1 more source
Identification of altered immune landscape at single-cell resolution in NSCLC brain metastasis and its association with poor immune checkpoint inhibitor responses. [PDF]
Bai M +14 more
europepmc +1 more source
Proteostasis of organelles in aging and disease
Cells rely on regulated proteostasis mechanisms to keep their internal compartments functioning properly. When these mechanisms fail, damaged proteins accumulate, disrupting organelles, such as the nucleus, mitochondria, endoplasmic reticulum, Golgi, and lysosomes, as well as membraneless organelles, such as stress granules, processing bodies, the ...
Yara Nabawi +5 more
wiley +1 more source
Anisosmotic Modulation of Mutant Huntingtin Aggregation vis-a-vis HSP70 Induction─Implications for Aging, Hypo-Hydration, and Neurodegeneration. [PDF]
Liu AYC, Kwan KY, Kwan C, Chen KY.
europepmc +1 more source
In this issue, Elmaleh et al. demonstrate that the E425K mutation in DNAJC7, associated with amyotrophic lateral sclerosis, selectively abolishes one of a co‐chaperone's dual functions. High‐resolution NMR confirmed that the mutation leaves the protein's structure intact while paralyzing its communication with the Hsp70 machinery.
Tsung‐Sheng Chiang +2 more
wiley +1 more source
Structures of the 26<i>S</i> proteasome in complex with the Hsp70 co-chaperone Bag1 reveal a mechanism for direct substrate transfer. [PDF]
Maestro-López M +10 more
europepmc +1 more source

