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Gene expression profile of spinal motor neurons in sporadic amyotrophic lateral sclerosis
TLDR
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*
TLDR
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
Loss of TDP-43 causes age-dependent progressive motor neuron degeneration.
TLDR
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
Disulfide Bond Mediates Aggregation, Toxicity, and Ubiquitylation of Familial Amyotrophic Lateral Sclerosis-linked Mutant SOD1*
TLDR
Dorfin ubiquitylated mutant SOD1 by recognizing the Cys6- and Cys111-disulfide cross-linked form and targeted it for proteasomal degradation, thereby promoting its degradation. Expand
The potential of GPNMB as novel neuroprotective factor in amyotrophic lateral sclerosis
TLDR
Glycoprotein nonmetastatic melanoma protein B can be a target for therapeutic intervention for suppressing motor neuron degeneration in ALS, and expression was substantial in the sera of sporadic ALS patients than that of other diseased patients. Expand
Calcium-sensing Receptor Ubiquitination and Degradation Mediated by the E3 Ubiquitin Ligase Dorfin*
TLDR
Because endogenous CaR in Madin-Darby canine kidney cells is also subject to degradation from the endoplasmic reticulum, dorfin-mediated ubiquitination may contribute to a general mechanism for CaR quality control during biosynthesis. Expand
Differentially expressed genes in sporadic amyotrophic lateral sclerosis spinal cords – screening by molecular indexing and subsequent cDNA microarray analysis
TLDR
Results indicated that genes associated with the ubiquitin–proteasome system, oxidative toxicity, transcription, neuronal differentiation and inflammation might be involved in the pathogenesis of SALS. Expand
Dorfin localizes to the ubiquitylated inclusions in Parkinson's disease, dementia with Lewy bodies, multiple system atrophy, and amyotrophic lateral sclerosis.
TLDR
This work reports that affinity-purified anti-Dorfin antibody labeled ubiquitylated inclusions of Parkinson's disease, dementia with Lewy bodies disease, multiple system atrophy, and sporadic and familial ALS show a distribution pattern parallel to that of ubiquitin, and suggests that Dorfin plays a crucial role in the formation of aggregates of alpha-synucleinopathy and ALS. Expand
Tuft-shaped astrocytes in Lewy body disease
TLDR
The findings suggest that in a subgroup of elderly individual cases, especially associated with LB pathology, the glial and neuronal p-tau accumulation is increased and has a distributional pattern similar to PSP. Expand
Oxidative stress induced by glutathione depletion reproduces pathological modifications of TDP-43 linked to TDP-43 proteinopathies
TLDR
It is shown that ethacrynic acid (EA), which is able to increase cellular oxidative stress through glutathione depletion, induces TDP-43 C- terminal phosphorylation at serine 403/404 and 409/410, insolubilization, C-terminal fragmentation, and cytoplasmic distribution in NSC34 cells and primary cortical neurons. Expand
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