Subject collection: Neurodegenerative Disorders
- 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.
- Inhibition of the IGF-1–PI3K–Akt–mTORC2 pathway in lipid rafts increases neuronal vulnerability in a genetic lysosomal glycosphingolipidosis
Summary: The authors present a mechanistic model to understand how alterations of the membrane architecture by the progressive accumulation of sphingolipids undermines basic cell function, with potential implications for a variety of genetic sphingolipidoses and adult neurodegenerative conditions.
- Amyotrophic lateral sclerosis mutant TDP-43 may cause synaptic dysfunction through altered dendritic spine function
Summary: Loss of synaptic GluR1, and reduced excitability within pyramidal neurons, implicates hypoexcitability and attenuated synaptic function in the pathogenic decline of neuronal function in TDP-43-associated ALS.
- Recapitulating Parkinson's disease pathology in a three-dimensional human neural cell culture model
Editor's choice: This study describes a novel 3D cell culture model that recapitulates key features of neuropathology in synucleinopathies, including large, nuclear-associated α-synuclein and ubiquitin-positive inclusions.
- VPS13A is closely associated with mitochondria and is required for efficient lysosomal degradation
Summary: VPS13A, which is implicated in chorea-acanthocytosis, is essential for efficient lysosomal degradation, while localized in close association with mitochondria. We propose that inter-organelle communication may be relevant in the pathogenesis of the disease.
- ApoE-associated modulation of neuroprotection from Aβ-mediated neurodegeneration in transgenic Caenorhabditis elegans
Summary: We report new humanized nematode models of amyloid-beta and apolipoprotein E gene expression, examining readouts for neurodegeneration, behavior, cellular function and survival, to elucidate the relative contributions of the proteins to the underlying pathology of Alzheimer's disease.