Zhefan Stephen Chen
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View article: Mutant huntingtin induces neuronal apoptosis via derepressing the non-canonical poly(A) polymerase PAPD5
Mutant huntingtin induces neuronal apoptosis via derepressing the non-canonical poly(A) polymerase PAPD5 Open
MicroRNAs (miRNAs) are small non-coding RNAs that play crucial roles in post-transcriptional gene regulation. Poly(A) RNA polymerase D5 (PAPD5) catalyzes the addition of adenosine to the 3′ end of miRNAs. In this study, we demonstrate that…
View article: Mutant huntingtin induces neuronal apoptosis via derepressing the non-canonical poly(A) polymerase PAPD5
Mutant huntingtin induces neuronal apoptosis via derepressing the non-canonical poly(A) polymerase PAPD5 Open
MicroRNAs (miRNAs) are small non-coding RNAs responsible for RNA silencing and the posttranscriptional regulation of gene expression. Poly(A) RNA polymerase D5 (PAPD5) catalyzes the addition of adenosine to the 3′ end of miRNAs, which prom…
View article: Upregulation of FasII underlies synergistic neuropathological and behavioral defects in a<i>Drosophila</i>model of myotonic dystrophy
Upregulation of FasII underlies synergistic neuropathological and behavioral defects in a<i>Drosophila</i>model of myotonic dystrophy Open
Myotonic dystrophy type 1 (DM1) is a multisystemic disorder that has been extensively studied for decades, yet our understanding of its neuropathological aspect remains rudimentary. In this study, we characterized a novel model of DM1 neur…
View article: Mutant GGGGCC RNA prevents YY1 from binding to Fuzzy promoter which stimulates Wnt/β-catenin pathway in C9ALS/FTD
Mutant GGGGCC RNA prevents YY1 from binding to Fuzzy promoter which stimulates Wnt/β-catenin pathway in C9ALS/FTD Open
The GGGGCC hexanucleotide repeat expansion mutation in the chromosome 9 open reading frame 72 ( C9orf72 ) gene is a major genetic cause of amyotrophic lateral sclerosis and frontotemporal dementia (C9ALS/FTD). In this study, we demonstrate…
View article: Multifaceted investigation underlies diverse mechanisms contributing to the downregulation of Hedgehog pathway-associated genes INTU and IFT88 in lung adenocarcinoma and uterine corpus endometrial carcinoma
Multifaceted investigation underlies diverse mechanisms contributing to the downregulation of Hedgehog pathway-associated genes INTU and IFT88 in lung adenocarcinoma and uterine corpus endometrial carcinoma Open
Hedgehog (Hh) signaling primarily functions in the control of mammalian embryonic development but also has roles in cancer. The Hh activation depends on ciliogenesis, a cellular process that describes outgrowth of the primary cilium from c…
View article: Molecular insights into the interaction of CAG trinucleotide RNA repeats with nucleolin and its implication in polyglutamine diseases
Molecular insights into the interaction of CAG trinucleotide RNA repeats with nucleolin and its implication in polyglutamine diseases Open
Polyglutamine (polyQ) diseases are a type of inherited neurodegenerative disorders caused by cytosine–adenine–guanine (CAG) trinucleotide expansion within the coding region of the disease-associated genes. We previously demonstrated that a…
View article: A peptide inhibitor that rescues polyglutamine-induced synaptic defects and cell death through suppressing RNA and protein toxicities
A peptide inhibitor that rescues polyglutamine-induced synaptic defects and cell death through suppressing RNA and protein toxicities Open
Polyglutamine (polyQ) diseases, including spinocerebellar ataxias and Huntington's disease, are progressive neurodegenerative disorders caused by CAG triplet-repeat expansion in the coding regions of disease-associated genes. In this study…
View article: Recent Technologies for the Extraction and Separation of Polyphenols in Different Plants: A Review
Recent Technologies for the Extraction and Separation of Polyphenols in Different Plants: A Review Open
Polyphenol is an important secondary metabolite with unique physiological functions and biological activity. The polyphenols in different plants and biomass have different chemical structures, which needs various extraction methods to obta…
View article: Insight in the Recent Application of Polyphenols From Biomass
Insight in the Recent Application of Polyphenols From Biomass Open
Biomass polyphenols are bio-active macromolecules with distinct chemical structures in a variety of biomass. In recent years, the study of biomass polyphenols and their application in food and medicine fields has become a research hotspot,…
View article: CAG RNAs induce DNA damage and apoptosis by silencing <i>NUDT16</i> expression in polyglutamine degeneration
CAG RNAs induce DNA damage and apoptosis by silencing <i>NUDT16</i> expression in polyglutamine degeneration Open
Significance Small CAG (sCAG) RNAs are neurotoxic, but their role in polyglutamine degeneration remains to be fully elucidated. We observed that cellular expression of sCAGs is sufficient to induce neuronal DNA damage and discovered that t…
View article: Pan-cancer investigation reveals mechanistic insights of planar cell polarity gene Fuz in carcinogenesis
Pan-cancer investigation reveals mechanistic insights of planar cell polarity gene Fuz in carcinogenesis Open
The fuzzy planar cell polarity protein (Fuz) is an effector component of the planar cell polarity (PCP) signaling. Together with other core and effector proteins, the PCP pathway controls polarized cell movements. Fuz was also reported as …
View article: A heterozygous mutation in the CCDC88C gene likely causes early-onset pure hereditary spastic paraplegia: a case report
A heterozygous mutation in the CCDC88C gene likely causes early-onset pure hereditary spastic paraplegia: a case report Open
View article: A fine balance between Prpf19 and Exoc7 in achieving degradation of aggregated protein and suppression of cell death in spinocerebellar ataxia type 3
A fine balance between Prpf19 and Exoc7 in achieving degradation of aggregated protein and suppression of cell death in spinocerebellar ataxia type 3 Open
View article: Isolation and Identification of a Novel Anti-protein Aggregation Activity of Lignin-Carbohydrate Complex From Chionanthus retusus Leaves
Isolation and Identification of a Novel Anti-protein Aggregation Activity of Lignin-Carbohydrate Complex From Chionanthus retusus Leaves Open
Lignin-carbohydrate complex (LCC) is the biological macromolecule that has been demonstrated to exert multiple biological functions, including antioxidant, anti-inflammation and anti-tumorigenesis, which support its broad application in th…
View article: A Peptidylic Inhibitor for Neutralizing (GGGGCC)-Associated Neurodegeneration in C9ALS-FTD
A Peptidylic Inhibitor for Neutralizing (GGGGCC)-Associated Neurodegeneration in C9ALS-FTD Open
View article: FipoQ/<scp>FBXO</scp>33, a Cullin‐1‐based ubiquitin ligase complex component modulates ubiquitination and solubility of polyglutamine disease protein
FipoQ/<span>FBXO</span>33, a Cullin‐1‐based ubiquitin ligase complex component modulates ubiquitination and solubility of polyglutamine disease protein Open
Polyglutamine (polyQ) diseases describe a group of progressive neurodegenerative disorders caused by the CAG triplet repeat expansion in the coding region of the disease genes. To date, nine such diseases, including spinocerebellar ataxia …
View article: Transcriptional dysregulation in neurodegenerative diseases: Who tipped the balance of Yin Yang 1 in the brain?
Transcriptional dysregulation in neurodegenerative diseases: Who tipped the balance of Yin Yang 1 in the brain? Open
Yin Yang 1 (YY1) is a multi-functional transcription factor that regulates gene expression in a range of cell types, including neurons. It controls neuronal differentiation, as well as neuronal specification and migration during the develo…
View article: AQAMAN, a bisamidine-based inhibitor of toxic protein inclusions in neurons, ameliorates cytotoxicity in polyglutamine disease models
AQAMAN, a bisamidine-based inhibitor of toxic protein inclusions in neurons, ameliorates cytotoxicity in polyglutamine disease models Open
Polyglutamine (polyQ) diseases are a group of dominantly inherited neurodegenerative disorders caused by the expansion of an unstable CAG repeat in the coding region of the affected genes. Hallmarks of polyQ diseases include the acc…
View article: <i>Drosophila</i> Exo70 Is Essential for Neurite Extension and Survival under Thermal Stress
<i>Drosophila</i> Exo70 Is Essential for Neurite Extension and Survival under Thermal Stress Open
The octomeric exocyst complex governs the final step of exocytosis in both plants and animals. Its roles, however, extend beyond exocytosis and include organelle biogenesis, ciliogenesis, cell migration, and cell growth. Exo70 is a conserv…
View article: Planar cell polarity gene <i>Fuz</i> triggers apoptosis in neurodegenerative disease models
Planar cell polarity gene <i>Fuz</i> triggers apoptosis in neurodegenerative disease models Open
View article: A peptidylic inhibitor for neutralizing expanded<i>CAG</i>RNA-induced nucleolar stress in polyglutamine diseases
A peptidylic inhibitor for neutralizing expanded<i>CAG</i>RNA-induced nucleolar stress in polyglutamine diseases Open
Polyglutamine (polyQ) diseases are a class of progressive neurodegenerative disorders characterized by the expression of both expanded CAG RNA and misfolded polyQ protein. We previously reported that the direct interaction between expanded…