神经生物学

Neuronal cultures, including motoneuron (MN) cultures, are established from embryonic animals. These approaches have provided novel insights into developmental and possibly disease mechanisms mediating cell survival or death. Motoneurons isolated from mouse models of di ...

Most neurodegenerative disorders are associated with aggregates of ubiquitinated proteins, such as Lewy bodies in Parkinson’s disease and neurofibrillary tangles in Alzheimer’s disease. Although the etiology of the sporadic forms of these disorders remains elusive, these ob ...

The great potential of induced pluripotent cells (iPS) cells is that it allows the possibility of deriving pluripotent stem cells from any human patient. Generation of patient-derived stem cells serves as a great source for developing cell replacement therapies and also for creating hum ...

In recent years, the rapid advances in human genetics have identified many genes that are responsible for inherited diseases. In the effort to understand pathogenic pathways and develop therapeutics, researchers have developed genetic animal models as critical tools for the purpose ...

Although the zebrafish (Danio rerio) has been used extensively for many years in neurodevelopmental studies, use of this teleost to study neurological diseases has evolved only recently. Being a vertebrate, this animal offers advantages for the study of human disease over other small ani ...

Ischemic stroke is among the leading causes of mortality and long-term disability in the western world. Despite enormous research activities in the last decades, current therapeutic options for acute stroke patients are still very limited. Reliable and realistic in vivo animal models r ...

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 ...