Subject collection: Neurodegenerative Disorders
- Establishment and validation of an endoplasmic reticulum stress reporter to monitor zebrafish ATF6 activity in development and disease
Summary: In this study, we validate transgenic zebrafish generated to specifically report the activity of ATF6, representing a major branch of the endoplasmic reticulum stress pathway with functions in development and disease.
- Misfolded α-synuclein causes hyperactive respiration without functional deficit in live neuroblastoma cells
Summary: Misfolded α-synuclein produced using the protein misfolding cyclic amplification assay associates with cardiolipin and causes hyperactive respiration in neuronal cells.
- Transgenic minipig model of Huntington's disease exhibiting gradually progressing neurodegeneration
Summary: Longitudinal phenotyping of the minipig model for Huntington's disease demonstrates a slow and age-dependent neurodegeneration.
- Longitudinal study revealing motor, cognitive and behavioral decline in a transgenic minipig model of Huntington's disease
Summary: The transgenic minipig model of Huntington's disease demonstrates a slow-progressing motor, cognitive and behavioral phenotype with later onset in adulthood.
- Quantitative whole-brain 3D imaging of tyrosine hydroxylase-labeled neuron architecture in the mouse MPTP model of Parkinson's disease
Summary: Whole-brain immunolabeling, mapping and absolute quantification of tyrosine hydroxylase neurons in the adult mouse brain provides a useful tool for studying changes in dopaminergic signaling in a mouse model of PD.
- Insoluble Aβ overexpression in an App knock-in mouse model alters microstructure and gamma oscillations in the prefrontal cortex, affecting anxiety-related behaviours
Summary: Aβ overexpression in a second-generation Alzheimer's disease model results in alterations in anxiety-type behaviours, as well as gamma oscillations, neural organisation and Grin2b expression in the prefrontal cortex.
- C9ORF72-related cellular pathology in skeletal myocytes derived from ALS-patient induced pluripotent stem cells
Summary: Evidence of protein aggregation and mitochondrial dysfunction were found in skeletal myocytes differentiated from ALS-patient induced pluripotent stem cells with the C9ORF72 mutation.
- Vascular regression precedes motor neuron loss in the FUS (1-359) ALS mouse model
Summary: Vascular regression is observed prior to motor neuron loss in the FUS (1-359) mouse model of ALS, yet is not rescued by angiogenin treatment.
- Sensory neuropathy and nociception in rodent models of Parkinson's disease
Summary: Rodent models of Parkinson's disease partially develop prodromal somatosensory and olfactory dysfunctions reminiscent of sensory neuropathies in patients and reveal mechanistic insight, but data are incomplete and fragmented.
- Effects of subthalamic deep brain stimulation on striatal metabolic connectivity in a rat hemiparkinsonian model
Summary: Deep brain stimulation in the subthalamic nucleus in rats with a unilateral dopaminergic lesion established a new functional interhemispheric striatal network.