Inducible expression of human C9ORF72 36× G4C2 hexanucleotide repeats is sufficient to cause RAN translation and rapid muscular atrophy in mice
FW Riemslagh, EC van der Toorn… - Disease models & …, 2021 - journals.biologists.com
The hexanucleotide G 4 C 2 repeat expansion in the first intron of the C9ORF72 gene accounts
for the majority of frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS) …
for the majority of frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS) …
[HTML][HTML] Lack of a clear behavioral phenotype in an inducible FXTAS mouse model despite the presence of neuronal FMRpolyG-positive aggregates
…, V Boumeester, EC Van der Toorn… - Frontiers in Molecular …, 2020 - frontiersin.org
Fragile X-associated tremor/ataxia syndrome (FXTAS) is a rare neurodegenerative disorder
caused by a 55–200 CGG repeat expansion in the 5′ untranslated region of the Fragile X …
caused by a 55–200 CGG repeat expansion in the 5′ untranslated region of the Fragile X …
Reduction of oxidative stress suppresses poly-GR-mediated toxicity in zebrafish embryos
…, RFM Verhagen, EC van der Toorn… - Disease Models & …, 2021 - journals.biologists.com
The hexanucleotide (G 4 C 2 )-repeat expansion in the C9ORF72 gene is the most common
pathogenic cause of frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS). …
pathogenic cause of frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS). …
Neuropathology of FMR1-premutation carriers presenting with dementia and neuropsychiatric symptoms
…, NA Verwey, ND Prins, EC van der Toorn… - Brain …, 2021 - academic.oup.com
… Esmay C van der Toorn , Esmay C van der Toorn … The authors thank the Netherlands
Brain Bank for providing tissue and Irene van Dijken for her input on the immunofluorescence …
Brain Bank for providing tissue and Irene van Dijken for her input on the immunofluorescence …
A missense variant in the nuclear export signal of the FMR1 gene causes intellectual disability
S Zeidler, LA Severijnen, H de Boer, EC van der Toorn… - Gene, 2021 - Elsevier
Fragile X syndrome (FXS) is the most common monogenetic cause of intellectual disability
and autism spectrum disorders. Mostly, FXS is caused by transcriptional silencing of the …
and autism spectrum disorders. Mostly, FXS is caused by transcriptional silencing of the …
Photoenzymatic reduction of C C double bonds
MM Grau, JC van der Toorn, LG Otten… - Advanced Synthesis …, 2009 - Wiley Online Library
… van der Toorn … van der Toorn … For the latter, so-called enoate reductases [EC 1.3.1.X] (or
old yellow enzymes, OYEs)6 have recently experienced a renewed interest as catalysts for the …
old yellow enzymes, OYEs)6 have recently experienced a renewed interest as catalysts for the …
Mitochondrial localization and function of heme oxygenase-1 in cigarette smoke–induced cell death
DJ Slebos, SW Ryter, M Van Der Toorn… - American journal of …, 2007 - atsjournals.org
Cigarette smoke–induced apoptosis and necrosis contribute to the pathogenesis of chronic
obstructive pulmonary disease. The induction of heme oxygenase-1 provides cytoprotection …
obstructive pulmonary disease. The induction of heme oxygenase-1 provides cytoprotection …
Cigarette smoke irreversibly modifies glutathione in airway epithelial cells
M van der Toorn, MP Smit-de Vries… - … of Physiology-Lung …, 2007 - journals.physiology.org
… EC 50 values of a variety of aldehydes that are able to deplete GSH showed that acrolein
was by far the most effective one to bind to GSH followed by formaldehyde and …
was by far the most effective one to bind to GSH followed by formaldehyde and …
RF-noise modeling in advanced CMOS technologies
…, LF Tiemeijer, R van der Toorn… - … on Electron Devices, 2013 - ieeexplore.ieee.org
… Moreover, the “critical field” is given by Ec ∼ vth/μ0, while in the case of velocity saturation
we have Ec ∼ vsat/μ0. Unfortunately, the thermal velocity vth at room temperature has almost …
we have Ec ∼ vsat/μ0. Unfortunately, the thermal velocity vth at room temperature has almost …
Cigarette smoke-induced blockade of the mitochondrial respiratory chain switches lung epithelial cell apoptosis into necrosis
M van der Toorn, DJ Slebos… - … of Physiology-Lung …, 2007 - journals.physiology.org
Increased lung cell apoptosis and necrosis occur in patients with chronic obstructive pulmonary
disease (COPD). Mitochondria are crucially involved in the regulation of these cell death …
disease (COPD). Mitochondria are crucially involved in the regulation of these cell death …