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ProteomicsUBXN1 maintains ER proteostasis and represses UPR activation by modulating translation
ER protein homeostasis (proteostasis) is essential to facilitate proper folding and maturation of proteins in the secretory pathway. Loss of ER proteostasis can lead to the accumulation of misfolded or aberrant proteins in the ER and triggers the unfolded protein response (UPR). Here we find that th...
ORGANISM(S): Homo sapiens (Human) 
2024-06-16 | PXD043686 | Pride
Ubxn Tmt Upr Er stress Proteostasis 
ProteomicsALS-related p97 R155H mutation disrupts lysophagy in iPSC-derived motor neurons
Mutations in the AAA+ ATPase p97 cause multisystem proteinopathy 1 which includes amyotrophic-lateral sclerosis; however, the pathogenic mechanisms that contribute to motor neuron loss remain obscure. Here, we use two induced pluripotent stem cell models differentiated into spinal motor neurons to i...
ORGANISM(S): Mus musculus (Mouse) Homo sapiens (Human) 
2025-09-08 | PXD048185 | Pride
Ubxd1 Lysosome Human motor neurons Cb-5083 P97 
ProteomicsThe p97-UBXD8 complex regulates ER-Mitochondria contact sites by altering membrane lipid saturation and composition
The intimate association between the endoplasmic reticulum (ER) and mitochondrial membranes at ER-mitochondria contact sites (ERMCS) serves as a platform for several critical cellular processes, particularly lipid synthesis. How contacts are remodeled and the subsequent biological consequences of al...
ORGANISM(S): Homo sapiens (Human) 
2023-01-25 | PXD039061 | Pride
Srebp1 Ermcs Scd1 Ubxd8 Neurodegeneration 
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