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Thioredoxin-interacting protein mediates ER stress-induced β cell death through initiation of the inflammasome.
It is reported that thioredoxin-interacting protein (TXNIP) is a critical signaling node that links ER stress and inflammation and is a potential therapeutic target for diabetes and ER stress-related human diseases such as Wolfram syndrome. Expand
Regulation of insulin biosynthesis in pancreatic beta cells by an endoplasmic reticulum-resident protein kinase IRE1.
IRE1 signaling activated by transient exposure to high glucose uses a unique subset of downstream components and has a beneficial effect on pancreatic beta cells, and is a potential target for therapeutic regulation of insulin biosynthesis. Expand
Wolfram syndrome 1 gene negatively regulates ER stress signaling in rodent and human cells.
A role for WFS1 is revealed in the negative regulation of ER stress signaling and in the pathogenesis of diseases involving chronic, unresolvable ER stress, such as pancreatic beta cell death in diabetes. Expand
Position-dependent FUS-RNA interactions regulate alternative splicing events and transcriptions
The analysis revealed that FUS regulates alternative splicing events and transcriptions in a position-dependent manner and binding of FUS to the promoter antisense strand downregulates transcriptions of the coding strand. Expand
Differential expression of inflammation‐ and apoptosis‐related genes in spinal cords of a mutant SOD1 transgenic mouse 
model of familial amyotrophic lateral sclerosis
Familial amyotrophic lateral sclerosis (FALS)‐linked mutations in copper–zinc superoxide dismutase (SOD1) cause motor neuron death through one or more acquired toxic properties. We analyzed theExpand
Gene expression profile of spinal motor neurons in sporadic amyotrophic lateral sclerosis
The motor neuron–specific gene expression profile in sporadic ALS can provide direct information on the genes leading to neurodegeneration and neuronal death and are helpful for developing new therapeutic strategies. Expand
Dorfin Ubiquitylates Mutant SOD1 and Prevents Mutant SOD1-mediated Neurotoxicity*
It is reported that Dorfin, a RING finger-type E3 ubiquitin ligase, is predominantly localized in the inclusion bodies of familial ALS with a copper/zinc superoxide dismutase (SOD1) mutation as well as sporadic ALS and protects neurons by recognizing and then ubiquitylating mutant SOD1 proteins followed by targeting them for proteasomal degradation. Expand
Position-specific binding of FUS to nascent RNA regulates mRNA length.
CAGE-seq and PolyA-seq revealed that position-specific regulation of mRNA lengths by FUS is operational in two-thirds of transcripts in neuronal cells, with enrichment in genes involved in synaptic activities. Expand
Loss of TDP-43 causes age-dependent progressive motor neuron degeneration.
It is suggested that transactive response DNA-binding protein 43 plays an essential role in the long term maintenance of motor neurons and that loss-of-function of this protein seems to contribute to the pathogenesis of amyotrophic lateral sclerosis. Expand
FUS/TLS assembles into stress granules and is a prosurvival factor during hyperosmolar stress
It is demonstrated that endogenous F US exerts a robust response to hyperosmolar stress induced by sorbitol, indicating a prosurvival role for endogenous FUS in the cellular response tohyperosmolars stress. Expand