Neurodegenerative disorders, such as amyotrophic lateral sclerosis (ALS), Huntington’s (HD), Parkinson’s (PD) and Alzheimer’s diseases (AD), are characterized by the loss of structure and function of specific neuronal circuitry in the brain. As a result of this loss, behavioral sympt ...
Parkinson’s disease (PD) is one of the most common neurodegenerative disorders. Despite the substantial progress that has been achieved, the precise mechanisms involved in the development of this disease are still not fully understood. The most common concepts relate to the genetic bac ...
Neurodegenerative diseases represent one of the most devastating types of diseases in older populations in our time. Significant efforts have been made over the last 20 years to understand the molecular, biochemical, and physiological alterations underlying these diseases. Howe ...
Ongoing investigations into causes and cures for human movement disorders are important toward the elucidation of diseases, such as Parkinson’s disease (PD). The use of animal model systems can provide links to susceptibility factors as well as therapeutic interventions. In this rega ...
The extracellular accumulation of insoluble fibrillar peptides in brain parenchyma and vessel walls as amyloid is the hallmark of neurodegenerative diseases, such as Alzheimer’s disease and Prion diseases. Regardless their amino acid sequences, all amyloid peptides adopt an ins ...
Protein aggregates/inclusions are pathological hallmarks of a wide spectrum of neurodegenerative �diseases. These aggregates have different shapes, sizes, distribution, and protein composition, which are unique features used for pathological diagnosis. The aggregat ...
The ubiquitin/proteasome pathway (UPP) is the major proteolytic quality control system in cells and involves tightly regulated removal of unwanted proteins and retention of those that are essential. In addition to its function in normal protein degradation, the UPP plays a critical role ...
Changes in intracellular calcium concentration play a major role both in signal transduction and in cell death. In particular, mitochondrial Ca2+ overload is critically important as a determinant of irreversible cell injury. When accumulated above a threshold, matrix Ca2+ triggers o ...
Proper control of mitochondrial turnover is critical for maintenance of cellular energetics under basal and stressed conditions, and for prevention of endogenous oxidative stress. Whole organelle turnover is mediated through macroautophagy, a process by which autophagoso ...
Recent discoveries have brought mitochondria functions in focus of the neuroscience research community and greatly stimulated the demand for approaches to study mitochondria dysfunction in neurodegenerative diseases. Many mouse disease models have been generated, but stu ...
Microarray-based assays have significantly expanded their scope and range of applications over the last 10 years, and – at least for gene expression – can be considered mainstream applications. High-throughput, microarray-based gene expression studies have proven particularly ...
The advent of viral gene therapy technology has contributed greatly to the study of a variety of medical conditions, and there is increasing promise for clinical translation of gene therapy into human treatments. Adeno-associated viral (AAV) vectors provide one of the more promising appr ...
RNA interference (RNAi) is a conserved cellular mechanism in most eukaryotes that can mediate specific gene silencing. Since its discovery in 1998, rapid progress has been made in understanding its basic mechanism and its application in research and drug discovery. In recent years, the app ...
Amyotrophic lateral sclerosis (ALS), a disorder that affects 30,000 individuals in the USA alone, is characterized by relatively rapid degeneration of upper and lower motor neurons, with death normally occurring 2–5 years following diagnosis due to respiratory paralysis. Transpl ...
Various neurodegenerative diseases are associated with aberrant gene expression. We recently identified a novel class of pimelic o-aminobenzamide histone deacetylase (HDAC) inhibitors that show promise as therapeutics in the neurodegenerative diseases Friedreich’s a ...
Parkinson’s disease (PD) is a neurological disorder caused by progressive degeneration of dopaminergic neurons in the nigrostriatal area of the brain. The decrease in dopamine (DA) neurotransmitter levels in the striatum and substantia nigra pars compacta is a neurochemistry hall ...
Emerging evidence suggests that synaptic dysfunction occurs prior to neuronal loss in neurodegenerative diseases, such as amyotrophic lateral sclerosis (ALS). Therefore, monitoring synaptic activity during early stages of neurodegeneration may provide valuable infor ...
Calcium is a ubiquitous intracellular messenger that has important functions in normal neuronal function. The pathology of Alzheimer’s disease has been shown to alter calcium homeostasis in neurons and astrocytes. Several calcium dye indicators are available to measure intrace ...
Optical imaging is a valuable tool for investigating alterations in membrane turnover and vesicle trafficking. Established techniques can easily be adapted to study the mechanisms of synaptic dysfunction in models of neuropsychiatric disorders and neurodegenerative dise ...
The voltage-gated sodium channel (Nav1) plays an important role in initiating and propagating action potentials in neuronal cells. We and others have recently found that the Alzheimer’s disease-related secretases BACE1 and presenilin (PS)/γ-secretase regulate Nav1 function by c ...